Chapter XXIII: Section VI: , Neurosyphilis and the War (5)

VASCULAR NEUROSYPHILIS[35] is illustrated in a fourth autopsied case. It may be noted that the pia mater in this case is practically normal. The tissues outside the area of softening due to the syphilitic thrombosis of nutrient vessels are practically normal. The case was one of almost complete sensory aphasia with word-deafness. The clinical picture is accordingly quite distinct from those of the paradigm (1) and of the case of general paresis (3) just discussed.

JUVENILE PARESIS[36] is illustrated by a case with exceedingly extensive lesions, largely meningeal and parenchymatous. The cerebral lesions are atypical since in places they suggest the tuberous sclerosis of Bourneville. The brain atrophy is extreme (965 grams) and it is possible that this apparent brain atrophy was in part hypoplasia, since the spirochetosis of this case was doubtless congenital. However, clinically the patient was fairly normal up to the age of 18.

A case of so-called SYPHILITIC EXTRAOCULAR PALSY[37] demonstrates a characteristic meningeal process more extensive than the clinical symptoms would have indicated. In fact, focal clinical nerve palsies are as a rule, if not constantly, partial phenomena of a far more extensive process of neurosyphilis. They are far more limited clinically than anatomically and histologically. It seems at first sight improper to term them cases of diffuse neurosyphilis in view of their clinical focality, yet they are best described as partial cases of diffuse neurosyphilis.

A case of GUMMA[38] of the left HEMISPHERE is presented which appears to have led to death in about four years from onset. This case, like many others, is not an example of purely focalized syphilitic process inasmuch as cysts of softening indicating slight vascular insults are present elsewhere (pons). There is also a degree of leptomeningitis, particularly basal.

Our discussion of the nature and forms of neurosyphilis is completed by a rare case probably belonging in the so-called _cervical hypertrophic meningitis of Charcot_ but actually due to a GUMMA OF THE SPINAL MENINGES.[39] The importance of therapeutic optimism is emphasized in this case as in the paradigm. Theoretically the meningeal inflammation of neurosyphilis ought to be almost entirely if not entirely removed by therapy, and these two cases, like several others in the series, seem to illustrate this possibility.

Neurosyphilis sometimes receives the clinical diagnosis neurasthenia simply through omission to apply proved diagnostic methods. An instance is given in which the PARETIC form of NEUROSYPHILIS (“general paresis”) received the diagnosis _neurasthenia_[40] for a period of five years, at any time during which period it would doubtless have been possible to render the correct diagnosis and apply treatment.

Neurosyphilis may imitate not only the psychoneuroses but also the psychoses themselves. We present a case of an architect, which looked almost precisely like _manic-depressive psychosis_[41] and had a history of attacks, but in which the positive serum W. R. led (in accordance with hospital rules) to an examination of the spinal fluid. The spinal fluid tests proved the case to be one of PARETIC NEUROSYPHILIS.

However, a positive serum W. R., even when associated with mental symptoms, and when those mental symptoms include grandiosity, does not prove the existence of neurosyphilis either in its paretic or non-paretic form. Our instance seems to be one of MANIC-DEPRESSIVE PSYCHOSIS.[42] The spinal fluid tests were entirely negative. The course of the disease was also that of manic-depressive psychosis. In the absence of positive spinal fluid tests, the diagnosis neurosyphilis was excluded.

Neurosyphilis and even PARETIC NEUROSYPHILIS may result in symptoms that would ordinarily lead to the diagnosis _dementia praecox_.[43]

It is important not to rule out neurosyphilis on the ground of a _negative serum_ W. R. The fluid W. R. may turn out positive. We present a case (of a salesman)[44] in which the serum W. R. was repeatedly negative (even salvarsan did not act provocatively) yet the spinal fluid W. R. proved positive. The case was clinically one of classical PARETIC NEUROSYPHILIS (“general paresis”). It is a good rule to proceed to lumbar puncture, even when the serum W. R. is negative, if there are suspicious symptoms (e.g., speech defect and memory impairment, grandiosity) or signs (e.g., marked reflex disorder, especially pupillary disorder).

DIFFUSE NEUROSYPHILIS was above defined as “meningovasculoparenchymatous.” This disease is typically associated with six positive tests (positive serum W. R., positive fluid W. R., pleocytosis, gold sol reaction, positive globulin reaction and excess albumin). One or more and frequently several of these six tests are likely to run mild in diffuse neurosyphilis; that is to say, these tests are apt to run milder than the identical tests in paretic neurosyphilis (“general paresis”). The clinical course of the diffuse, and especially the meningovascular cases, is likely to be protracted. The prognosis as to life is good, barring fatal vascular insults. The illustrative case[45] was a case with slow course. There was a series of attacks followed by a paralytic stroke, a finding highly typical of the diffuse form of neurosyphilis. The spinal fluid reactions were mild, suitable to the general principle above stated.

These tests are likely to run stronger, as above stated, in paretic neurosyphilis (“general paresis”), than in the diffuse form. In particular, the gold sol reaction is likely to be shown in what is termed “paretic” form rather than in what is termed “syphilitic” form. The clinical course of PARETIC NEUROSYPHILIS is likely to be brief. A characteristic case[46] with very heavy globulin and albumin tests is presented.

TABOPARETIC NEUROSYPHILIS[47] (“taboparesis”) is clinically a combination of the symptoms of tabetic (“tabes dorsalis”) and those of paretic neurosyphilis (“general paresis”). First comes the tabes dorsalis lasting often for many years. Afterward follows a characteristic general paresis. The ultimate paretic picture is likely to retain, however, various characteristics of tabes. The laboratory tests in the paretic phase of taboparesis are characteristic of general paresis and not of tabes dorsalis. The prognosis after the paretic phase has arrived is that of general paresis.

The diagnosis of the neurosyphilitic forms would be easy if these principles were always carried out to the letter. The important fact is as follows: diffuse (that is, meningovasculoparenchymatous neurosyphilis) may look like paretic neurosyphilis (“general paresis”)[48] at certain periods of the clinical and laboratory examination. This fact is of obvious importance. The general prognosis of diffuse neurosyphilis is regarded as good _quoad vitam_. The general prognosis of paresis is bad. If, however, the differential diagnosis cannot be rendered at particular phases of a given case, then no safe prognosis can be offered in the individual case. In particular no prognosis affecting the administration or non-administration of modern systematic treatment can or should be offered in these doubtful phases.

It is not always safe to exclude neurosyphilis even when the _fluid_ W. R. is _negative_.[49] Particularly in vascular neurosyphilis the fluid W. R. and even all the other laboratory signs in the spinal fluid may sometimes be negative. A positive serum W. R. yields the correct pointer to diagnosis. Of course, also in many cases of vascular neurosyphilis one or more of the laboratory signs may be suggestive even when the fluid W. R. is negative. Theoretically there may be cases in which all the six tests are negative and yet the diagnosis neurosyphilis be the correct one.

A clinically important sign in neurosyphilis is the so-called _seizures_. These occur both in DIFFUSE NON-PARETIC NEUROSYPHILIS[50] and in PARETIC NEUROSYPHILIS.[51]

_Aphasia_ is likewise a symptom in both these forms of neurosyphilis, namely, in the DIFFUSE non-paretic[52] and in the PARETIC form.[53]

The literature contains reference not only to seizures and aphasia as characteristically paretic but also to _remissions_. Remissions like seizures and aphasia are found in both the PARETIC[54] and NON-PARETIC forms of neurosyphilis.[55] They have important bearings on prognosis in all forms of neurosyphilis and are of especial significance in the evaluation of treatment. (Remissions coincident with apparent cure.)

So far we have been dealing with cases of neurosyphilis in which there was no doubt of the existence of mental symptoms. There are cases, however, in which although the laboratory signs of neurosyphilis exist, proving beyond doubt the existence of a chronic inflammatory reaction and allied pathological conditions in the cerebrospinal axis, there are no mental symptoms of neurosyphilis. We have called some of these cases PARESIS SINE PARESI[56] and present examples.

To illustrate complications we give a case of PARETIC NEUROSYPHILIS with autopsy in which there were ante mortem signs of HERPES ZOSTER[57] or, at all events, of a skin eruption limited to the area of a thoracic nerve.

A case of GUMMA of the brain[58] in which decompression was warranted and performed is presented. The fluid W. R., as in many such cases, was negative; serum positive.

A case of CRANIAL NEUROSYPHILIS (extraocular palsy[59] without mental symptoms) showed a positive Wassermann serum test and a negative spinal fluid.

The laboratory reactions in TABETIC NEUROSYPHILIS[60] (“tabes dorsalis”) run somewhat like those of diffuse non-paretic neurosyphilis and are accordingly milder than those of paretic neurosyphilis. The fluid W. R. and the gold sol reaction in particular are apt to run mild. The clinical course of tabes dorsalis is well known to be protracted and the prognosis _quoad vitam_ is good except that we must always bear in mind the possibility of vascular insults and complications of a syphilitic origin in the rest of the body.

It is important to remember that TABETIC NEUROSYPHILIS is often quite atypical[61] clinically and may even show no single symptom warranting the old clinical name locomotor ataxia.

There are even cases in which the name tabes dorsalis is not warranted in view of the fact that the lesions are not low in the cord but are higher up (TABES CERVICALIS[62]).

A rare form of neurosyphilis is ERB’S SYPHILITIC SPASTIC PARAPLEGIA[63] against which one needs to consider a number of non-syphilitic spinal cord diseases. Our case showed a weakly positive serum W. R., a negative fluid W. R., and the other tests of the spinal fluid were moderately positive.

SYPHILITIC MUSCULAR ATROPHY[64] is classified by Head and Fearnsides both in their meningovascular group and in their group of the so-called syphilis centralis. Our case affecting in large part the small muscles of the hands in a teamster, may be due either to spinal parenchymal lesions or to root neuritis or to both.

It is a little extraordinary and very important that the _laboratory signs_ are apt to be positive even in the SECONDARY period of SYPHILIS. Perhaps a third of all cases of syphilis in the secondaries would, if tested, yield positives precisely like those of full-blown paretic or diffuse neurosyphilis. Strangely enough, these signs may occur without clinical symptoms. The illustrative case,[65] a mechanic, yielded various mental symptoms. The cases of secondary syphilis with laboratory signs of neurosyphilis but without clinical symptoms are of the greatest theoretical importance in relation to the problem above mentioned of _paresis sine paresi_. It may well be inquired whether in some instances the neurosyphilis of the secondaries does not persist until the exhibition of mental or physical symptoms of neurosyphilis years later. It must be remembered that this conception is hardly more than a hypothesis at the present time. That such signs of chronic inflammation could exist without symptoms is not so surprising when one thinks of the startling immediate improvement seen after treatment or even in remissions without treatment. One is reminded of the crisis in pneumonia wherein clinical improvement takes place entirely independent of the mechanical conditions in the lung which just after the crisis remain as suppurative as before.

The diagnosis of JUVENILE NEUROSYPHILIS is made upon the same lines as that of neurosyphilis in the adult. We present two cases, one with optic atrophy[66] and the other with signs of congenital syphilis antedating the symptoms of paresis.[67]

Congenital syphilis is also apparently capable of producing a simple form of FEEBLEMINDEDNESS,[68] that is to say, a form of disease non-paretic, non-tabetic, and without special tendency to vascular insults.

We present a case of JUVENILE TABETIC NEUROSYPHILIS (“juvenile tabes”).[69] The tests were all positive.

The line of separation between typical and atypical cases of neurosyphilis is vague and indistinct and some of the cases classified by us amongst puzzles perhaps belong under systematic diagnosis and vice versa. The section on PUZZLES AND ERRORS in the diagnosis of neurosyphilis is introduced by six cases of error in the diagnosis of the paretic form of neurosyphilis.[70] These errors were made known by autopsy. Aside from the sixth case, whose etiology must remain in doubt and which was a unique case of PERIVASCULAR GLIOSIS, there is ground for the belief that the other five cases in this Danvers Hospital study of diagnostic errors were perhaps actually syphilitic though not of the paretic form of neurosyphilis. At all events, the brain tissues in these cases failed to show the plasma cell deposits which are characteristic in the sheaths of the intracortical vessels in paretic neurosyphilis.

A case illustrates the complication of TABES by _arteriosclerotic symptoms_, in which case the arteriosclerosis may naturally have been of syphilitic origin. Two cases especially illustrate the possibility of confusing the ataxia of general paresis with CEREBELLAR ATAXIA. These cases showed lesions of the cerebellar structures, notably of the dentate nucleus. No one can read these cases or any of the autopsied cases in our series, without perceiving how fundamental and even critical is the demand for autopsies in fatal cases of neurosyphilis. The practitioner who can secure an autopsy in a fatal case of neurosyphilis and have the tissues worked up by approved neuropathological methods is almost bound to add his bit to neurological theory. Even cases of classical tabes dorsalis are often signally important to the theorist on account of the relations of the neural to the non-neural complications.

We then proceed to a group of cases without special order in which a variety of diagnostic questions arose.

A case of questionable neurosyphilis in the secondary stage of syphilis brings up the problems of syphilitic _neurasthenia_.[71]

Syphilis may act as _agent provocateur_ of HYSTERIA as Charcot insisted.[72]

A case illustrative of difficulties in diagnosis between neurosyphilis and manic-depressive psychosis follows.[73]

A case for diagnosis is given which shows that errors in the diagnosis of neurosyphilis are entirely possible even when abundant clinical and laboratory data are available. A case with a weakly positive Wassermann reaction in the spinal fluid finally turned out to be one of BRAIN TUMOR.[74]

Some questions as to the diagnosis of NEUROSYPHILIS _versus Idiopathic Epilepsy_ are brought up by a case in which phenomena of paresis seemed to have occurred very early, about two years after the initial syphilitic infection.[75]

A case of PARETIC NEUROSYPHILIS is offered in which _hemiplegia_ and _hemitremor_ strongly suggested _vascular_ lesions; but the autopsy showed no coarse lesions and merely confirmed the diagnosis paresis microscopically.[76]

An autopsied case of PARETIC NEUROSYPHILIS is given, in which the pupils persisted in reacting normally. Herpes zoster-like lesions in life yielded no special signs at autopsy (all root-ganglia looked alike above and below zone of “shingles.”)[77]

An example of NEUROSYPHILIS, probably PARETIC, yielded symptoms highly suggestive of _manic-depressive psychosis_.[78] An interesting feature in this case was the birth of a healthy child nine months after the onset of the psychotic attack.

An example of _exophthalmic goitre_[79] following the acquisition of SYPHILIS showed at autopsy a heavy scarring of the optic thalamus and unilaterally atrophic process in the cerebral cortex.

We come to some questions concerning the _Argyll-Robertson pupil_. It is agreed on all hands that the Argyll-Robertson pupil is characteristic of the paretic and tabetic forms, but the sign occurs also in other neurosyphilitic conditions;[80] in fact the sign does not necessarily indicate neurosyphilis as an instance of PINEAL TUMOR demonstrates.[81]

The question raised above as to the possibility that neurosyphilis may exist in the absence of positive findings in the spinal fluid is illustrated in a man, a mechanic, who claimed syphilitic infection and showed an _Argyll-Robertson pupil_ on one side.[82] The serum W. R. was positive; the _fluid tests_ were _negative_.

An extraordinary case is given in some detail in which NEUROSYPHILIS in the form termed DISSEMINATED ENCEPHALITIS[83] proved fatal within seven months of the initial infection.

We have frequently mentioned the classical assumption that paretic neurosyphilis (“general paresis”) is a fatal disease. Some have suggested that there is another form clinically almost identical with general paresis except that it pursues a long course and the suggestion has been made that these cases be termed _pseudoparesis_.[84] We are of the opinion that this term should be dropped and advocate the use of the word pseudoparesis only for non-syphilitic disease looking like paresis, such as alcoholic pseudoparesis and the like.

The question whether there is a form of mental disease SYPHILITIC PARANOIA[85] is raised by a case with auditory hallucinations, ideas of persecution and attacks of excitement. The diagnosis of alcoholic hallucinosis was actually made although there is no proof that the patient ever drank alcohol.

Alcohol may cause symptoms identical with those of paretic neurosyphilis, including seizures, Argyll-Robertson pupils, speech defect and mental symptoms. The differentiation is readily made by the negative laboratory findings. An illustration is given in our case of the alcoholic teamster. Cases such as this bear the name ALCOHOLIC PSEUDOPARESIS.[86]

However, when the clinical picture is the same as in the case of our teamster, the alcohol may only be a complicating factor in neurosyphilis, as shown by our next case of the _alcoholic_ steamfitter who in fact was shown to have NEUROSYPHILIS.[87]

Sometimes cases of apparently frank _alcoholism_, even with apparently characteristic delirium tremens and neuritis, prove to be essentially neurosyphilitic.[88] On the other hand, true combinations of ALCOHOLISM and NEUROSYPHILIS occur which it would be proper to classify under either heading and in which therapy must take serious account of both conditions.[89]

As above stated, we elect to use the term pseudoparesis only for non-syphilitic cases. There are other forms of pseudoparesis than alcoholic pseudoparesis. The question of _Diabetic Pseudoparesis_ is raised by an exceedingly complicated case of which our best interpretation is that the patient, a proved syphilitic (with syphilitic osteomyelitis (?)), a huge doorkeeper, was perhaps suffering from an old SYPHILITIC scarring of the PITUITARY body.[90] Neither this case nor a second case, one of PARETIC NEUROSYPHILIS with _glycosuria_ is actually entitled to the diagnosis diabetic pseudoparesis. The second case of paretic neurosyphilis with glycosuria brings up some unanswerable questions as to the pancreatic or basal meningitic or other origin for the glycosuria.[91]

_Isolated symptoms_ are often presented by neurosyphilitics (e.g., hemianopsia);[92] but we tend to regard these cases as due to focal lesions that are merely part and parcel of DIFFUSE LESIONS.

A neurosyphilitic case (a steward) with the rather unusual complication (for our northern region) of severe MALARIA producing cerebral thrombosis is reported.[93]

The diagnosis _Dementia Praecox_[94] was actually made in the case of a young school-teacher in whom the laboratory findings proved conclusively that the condition was one of NEUROSYPHILIS. The gold sol reaction in this case was mild. The chief lesion at autopsy was a fresh looking, gelatinous pial exudate over the spinal cord which turned out to contain an almost pure display of very numerous plasma cells.

The question of LUES MALIGNA[95] is brought up in a rectifier of spirits in whom the characteristic tremendous destruction of tissue, toxemia and failure to react to antisyphilitic treatment were illustrated. Moreover, this case had a trauma (cautery) to the tonsil, as in other cases of lues maligna.

A case somewhat suggestive of _brain tumor_, of _neurosyphilis_ and of _multiple sclerosis_[96] turned out to be MULTIPLE SCLEROSIS (the fluid showed a pleocytosis and a moderate amount of globulin with a paretic type of gold sol reaction).

As a foil to this case that we regard as multiple sclerosis, we present a second case with nystagmus, optic atrophy and spasticity in which the suspicion of _multiple sclerosis_ might well be raised but which the tests demonstrated to be NEUROSYPHILITIC.[97]

An even stranger imitation of well-defined non-syphilitic entities was presented by a case apparently of _Huntington’s chorea_[98] (except for absence of the hereditary taint) which case, however, proved to the surprise of all diagnosticians to be one of NEUROSYPHILIS.

Frequent errors of diagnosis must occur in the field of the senile psychoses. We present a case that would at first blush warrant the diagnosis of _senile arteriosclerotic psychosis_[99] in a sea captain of 75 years (wife dead 15 years before of general paresis) who turned out to be a characteristic case from the laboratory standpoint of NEUROSYPHILIS.

The Protean nature of the symptomatology of neurosyphilis is sufficiently established. Still, a case that might fit into textbooks concerning DISSOCIATION OF PERSONALITY[100] is certainly a clinical oddity, as illustrated by a fugacious musician.

A case with strong suspicions of _neurosyphilis_ of _tabetic_ type turned out to be more probably one of neural complications in PERNICIOUS ANEMIA.[101]

NEUROSYPHILIS IN JUVENILES presents puzzling conditions.

One case was marked clinically by _attacks of excitement_.[102] It is impossible to place this case among the main groups of juvenile neurosyphilis.

Another case of FEEBLEMINDEDNESS,[103] also NEUROSYPHILITIC in origin, presented physical symptoms and laboratory signs of paretic neurosyphilis; yet this case had been considered one of _simple feeblemindedness_.

A case apparently of JUVENILE PARETIC NEUROSYPHILIS in a 15 year old boy presented the rather unusual complication of shocks with quadriplegia,[104] a _vascular complication_ not usually expected in the paretic type of neurosyphilis in adults.

Epileptic phenomena[105] are rare as the effect of JUVENILE NEUROSYPHILIS, but occur as demonstrated in a case which slipshod methods of diagnosis might well have regarded as one of _idiopathic epilepsy_.

A case of JUVENILE PARETIC NEUROSYPHILIS with the complication of ADDISON’S DISEASE[106] is given (autopsy confirmation).

The puzzle in diagnosis offered by syphilis in the secondary stage[107] is illustrated by a case which showed the characteristic NEUROSYPHILITIC complications of the SECONDARY STAGE of syphilis. This patient may well have been a moron at the outset and exhibited some reactions (refusal to talk) explicable on the basis of feeblemindedness. She was a neurosyphilitic only in the sense of the neural complication that we find in the secondary stage of syphilis. As stated above, we do not yet know what the fate of these neural complications of secondary syphilis is to be. The frequency of this finding in secondary syphilis is probably too great to warrant the hypothesis that it must always go on to a chronic neurosyphilis; but we certainly are warranted in regarding these cases as potential chronic neurosyphilitics.

A case of TABOPARETIC NEUROSYPHILIS in which the heavy exudate characteristic of paresis became a soil for a growth of the typhoid bacillus is presented with autopsy.[108] This fatality with TYPHOID MENINGITIS is merely a concrete example of the many complications which syphilitics and especially neurosyphilitics have to sustain.

The case series then goes on to illustrate, though quite inadequately, a variety of MEDICOLEGAL AND SOCIAL complications of neurosyphilis. It is well known that many social complications with grave moral, economic and even political difficulties occur.

Our series starts with a “public character”[109] whose eloquence and reformatory efforts led to a considerable notoriety. The autopsy in this case showed singularly few lesions despite the fact that the case was microscopically one of wholly characteristic PARETIC NEUROSYPHILIS. The question might arise how far we are entitled to correlate the reformatory efforts of this always eccentric character with syphilis. The man himself a physician, was aware of the doubt which his Argyll-Robertson pupils threw upon his medical situation. He explained them on the basis of an old smallpox! We are inclined to think that the whole of this man’s life, from his giving up of medical practice to live as a kind of literary and political hack, was due to subtle changes of neurosyphilitic origin. The fact that there was a certain delinquent streak in the man is not inconsistent with this idea. Interestingly enough, a fall on the ice in the man’s 61st year actually started up the fatal process, a condition of affairs amply illustrated in cases of neurosyphilis, brought out by trauma that come to the attention of the Industrial Accident Board in connection with claims for compensation.

A case of sudden _grandiosity_[110] illustrates an episode of NEUROSYPHILITIC origin. Such a person might well be regarded by the lay newspaper reader as a crank or a grafter but the neurosyphilitic possibility should always be entertained in cases of this order.

As against the social difficulties that look in the direction of the classical paretic grandeur, we present a case of apparent _suicidal attempt_ by gas, which attempt was followed by a period of amnesia that, taking into account the laboratory findings, was probably NEUROSYPHILITIC.[111]

Vistas of extraordinary interest are opened out by studies of the relation of neurosyphilis to _delinquency_. The case of the psychopathic reformer (Case 83) above mentioned was one in which the delinquency may possibly have been related to acquired syphilis. We present also a case of juvenile neurosyphilis, a young man of reform school type[112] in which JUVENILE PARETIC NEUROSYPHILIS was established. This patient, in fact, deteriorated very rapidly to a condition of considerable dementia a few months after the diagnosis was established.

A striking case of so-called DEFECTIVE DELINQUENCY is presented, an alcoholic prostitute of the reformatory group.[113] The NEUROSYPHILIS in this case was a complication rather than an original factor in the delinquency.

One case of PARESIS SINE PARESI was that of an habitual criminal[114] and forger who, without showing mental or physical symptoms of neurosyphilis, yielded the laboratory signs of paretic neurosyphilis. Again, as in the case of the prostitute just mentioned, the CRIMINALITY[115] seems to have antedated the neurosyphilis and even to have been hereditary.

By way of introducing the next group of Industrial Accident Board cases, we present a case of JUVENILE PARESIS with initial TRAUM.

The Industrial Board group is of note in that the signs of the traumatic form[116] of paretic neurosyphilis do not occur immediately upon the accident. Some time elapses in which the physical, chemical or parasitological changes have time to work themselves out in the injured tissues. Many hypotheses may be raised as to the reason why a trauma lights up a syphilitic process. Of course, =false claims=[117] =may be made for compensation by neurosyphilitics= in whom the symptoms were already in existence before the accident and in whom they may not even be markedly exacerbated by the accident. The false claimants can probably not readily frame a story which the expert psychiatrist cannot discredit if he is allowed to perform laboratory tests and give the patient the benefit of thorough examination. However, some cases of established PARETIC NEUROSYPHILIS are perhaps truly subject to _exacerbations_[118] of the clinical process and it may well be held that such exacerbations warrant partial compensation.

The fact that a trauma may light up a syphilitic process is illustrated in a case that came to the Psychopathic Hospital, in which a SYPHILITIC LESION developed in the skull AT THE SITE OF SKULL INJURY.[119]

A case of OCCUPATION-NEUROSIS[120] that might be interpreted as a _syphilitic neuritis_ is presented. The case is still in doubt as to its scientific evaluation.

The workmen’s compensation group of syphilitic cases is of extraordinary general interest since it indicates that employers may well be on the lookout not to employ known syphilitics unless fortified by special insurance arrangements. Whether in future employers may desire =to employ only W. R. negative workmen= is one of the highly complicated questions _re_ workmen’s compensation and health insurance.

But the problems of neurosyphilis are not merely medicolegal and broadly public or social. The most appealing difficulties lodge within the bosom of the family. Now and then a case of INCOMPATIBILITY OF TEMPERAMENT, perhaps complicated by _alcoholism_, occurs which tests prove to be NEUROSYPHILITIC.[121]

Special attention should be drawn to a certain NEUROSYPHILITIC FAMILY[122] in which both parents and five children showed a variety of syphilitic diseases, including syphilis without apparent neural complications, paretic neurosyphilis, juvenile paresis, aortic aneurysm, achondroplasia and caries of the spine, and an as yet indefinite neurosis. There was a sixth child that died shortly after birth, as well as three stillborn.

One =cannot conclude= from the normal[123] look of a neurosyphilitic’s family =that the normal-looking members are not syphilitic=, as illustrated by the family of our draughtsman.

The most =intricate social complications= may arise. We present a case of a syphilitic man (a well-to-do merchant) who was apparently being goaded into a second marriage[124] because he was continually being charged with having caused his first wife’s death. This he had actually done in a certain sense because his wife had died of general paresis, having contracted syphilis from him.

In the fifth section on THERAPY, we have attempted to outline some of the principles and problems that arise in the treatment of neurosyphilis. Enough has probably been said concerning the attitude of optimism or pessimistic nihilism that may be adopted toward the whole subject. It must be borne in mind, however, that a great deal of the work on treatment of neurosyphilis is still in the experimental stage. As a rule, each case must be considered separately and individually and the prognosis can be made satisfactorily only after treatment has been given. This section contains a group of cases that have been treated rather intensively and the results of this treatment are indicated. The section is introduced by _five untreated cases_, the brains and cords of which have been studied post mortem. These illustrate the pathological conditions which we have to meet, and from these examples we can draw the theoretical conclusion that some cases are beyond the aid of therapy on account of the brain destruction. Others, in which the symptomatology bespeaks just as grave a situation, turn out on autopsy to have very little actual damage to the brain tissues and therefore should theoretically at any rate be amenable to antisyphilitic therapy.

In order to get any adequate conception of the possibilities of therapeutic results in cases of neurosyphilis, one must consider the pathological changes that occur and how far these changes are reparable. In cases in which the destruction of tissue is marked, it is, of course, out of the question to expect to get any marked clinical improvement. A case of spastic hemiplegia[125] in paretic neurosyphilis is given with the autopsy findings as an illustration of irreparable damage that may occur to the parenchymatous structure, thus precluding any chance of functional recovery.

On the other hand, there is a group of cases in which the symptoms may be exceedingly severe and yet the actual destruction of tissue be almost nil. This point is illustrated by a case[126] in which _total duration of symptoms_ terminating in death was _only 22 days_. At autopsy there was very little in the way of macroscopical lesions, and microscopically there was no marked evidence of destruction in the parenchymatous tissue. The lesions were represented chiefly by perivascular infiltration. According to all our modern ideas, this type of reaction is resolvable under antisyphilitic treatment. Though this case was one of very short duration, similar pathological pictures may be obtained in cases of considerably longer standing. It is also of great importance to remember that symptomatically such a case may be in no way distinguished from a case with marked atrophy.

Another autopsied case is given which shows an exceedingly =marked meningitis=.[127] The meningitic processes according to the literature and experience react very readily to antisyphilitic treatment in the form either of mercury and iodid or in combination with salvarsan. The lesion here present would probably have improved had intensive treatment been given. Clinically the diagnosis of general paresis was made and, as has been the rule in the past, treatment was not given on the ground that it had no value in paresis. While this is an extreme case of meningitis, it is to be remembered that the vast majority of cases of paretic neurosyphilis show some degree of meningitis. Just as in the marked meningitis of the diffuse neurosyphilis, so with the meningitis of the paretic form, improvement is expected under treatment. As a part or even the whole of the symptomatology in a given case may be due to this meningitic process, we have reason occasionally to expect marked improvement as the result of antisyphilitic treatment.

As a contrast to this case with marked meningitis, another case of =marked atrophy=[128] is given. Here the atrophy was very perceptible on macroscopical examination and the mere view of the brain at once indicated that in such a case important results from treatment were not to be expected.

The =topographical variation= of the lesions in neurosyphilis must be remembered when treatment is to be instituted. Thus very marked lesions may exist in portions of the brain which do not give any very definite localizing symptoms. As a result, one may be led to believe from clinical evidence that the case is a very mild one though the lesions may really be very extensive. The topographical distribution must, therefore, be taken into consideration in trying to estimate the damage done. This point of topographical distribution of the lesions is illustrated by a case.[129]

It has been generally recognized that =clinical improvement=, if not cure, may be =readily obtained in the group of diffuse neurosyphilis=, i.e., so-called cerebral and cerebrospinal forms of syphilis. These are cases in which the parenchyma is very slightly, if at all, affected and in which the lesion is chiefly in the meninges and blood vessels, irritative rather than degenerative. A case[130] is given to illustrate this point. In our experience systematic intravenous salvarsan therapy associated with mercury and iodid gives remarkably good results in the vast majority of this group of cases.

It is generally conceded that antisyphilitic treatment, particularly salvarsan, has a very satisfactory result applied to diffuse neurosyphilis. But the same good results may be obtained in cases which are not so typically of the diffuse type. An illustration is given in the case of a machinist in which the diagnosis was in doubt between paretic, tabetic or diffuse neurosyphilis.[131] The result of treatment was as satisfactory as could be expected in any type of neurosyphilis and this in a case of several years’ duration with Argyll-Robertson pupils.

As a rule, the Argyll-Robertson pupil is taken as a grave omen for treatment, an idea based upon a conception that the Argyll-Robertson pupil so frequently represents the old so-called “parasyphilitic” cases, which, in the past were taught as being incapable of improvement by the ordinary antisyphilitic methods.

A second case[132] with Argyll-Robertson pupil shows again that the =prognosis may be very good despite the Argyll-Robertson sign=.

But even in the diffuse neurosyphilis, the symptomatic results of treatment may not be entirely happy. Under treatment it may be possible to reduce the spinal fluid tests to negative without, however, as in the case of our hemiplegic lady,[133] making the physical or mental symptoms disappear. In other words, it may be possible to stop the active progress of the disease without removing the symptoms.

One is always warned of the danger of intravenous salvarsan therapy in hemiplegic cases due to arteriosclerotic conditions. While this warning is well justified, it does not mean that the most intensive treatment is contraindicated, as shown in the case of our hemiplegic machinist.[134] Such may be given over long periods of time with the most satisfactory results.

A case[135] is given which illustrates the value of antisyphilitic treatment in cases showing symptoms of intracranial pressure due to syphilitic disease. In the case of the woman which we cite, we believe that the symptoms of intracranial pressure were probably due to a gummatous new growth, although it is possible that they were due to a marked meningitic process. However, the results of a limited amount of antisyphilitic treatment in this case were very brilliant. Similar results may often be obtained in gumma of the brain. This is not always true, however, and it may become necessary to use surgical procedure in order rapidly to overcome the effects of intracranial pressure.

While it has always been conceded that treatment would greatly help cases of diffuse and vascular neurosyphilis, the utmost pessimism has existed concerning the results to be obtained by treatment in cases of tabetic and paretic neurosyphilis. Only in the last five or six years, due to the stimulus of Ehrlich’s discovery of salvarsan and the introduction of the intraspinous methods of therapy, have intensive work and study been given to the treatment of these cases. And though it has been by no means settled in the minds of the various workers in this field, as to what the ultimate results of such treatment will be and though some do not believe that there is any good to be expected from our present methods, still the majority of men who are treating these cases systematically feel very much encouraged.

=At times very brilliant results= are to be obtained by intraspinous treatment =in tabetic neurosyphilis= (“tabes dorsalis”). A very striking illustration is given of a case of this sort in which the symptoms dated only a few months but which had all the classical symptoms, signs and laboratory tests. Five intraspinous injections of mercurialized serum were sufficient to cause the disappearance of the subjective symptoms and to reduce the spinal fluid test to negative.[136]

It must be emphasized that the best results in cases of tabetic neurosyphilis are usually to be expected in cases in which the symptoms are of short standing. Where the process is of long duration and much destruction of spinal cord tissue has occurred, the best one can expect is that the activity and progress may be halted. This is illustrated by our case of a baker, 43 years of age, who had been suffering from the symptoms of tabes for some years. Under treatment it was possible to get an entirely negative serology of the blood and spinal fluid.[137] Despite this evidence that the activity of syphilis had ceased, the symptoms continued unabated. We are ready to believe, however, that much good was accomplished. For the patient should not have any further untoward developments or the appearance of any new symptoms. These, without such treatment, might well be expected. At times excellent clinical results are obtained in long-standing cases.

The results of treatment in paretic neurosyphilis (“general paresis”) have been considered even less hopeful than in tabetic neurosyphilis (“tabes dorsalis”); indeed, it has often been stated that the patients are made worse by treatment. Recent work, however, supports a much more optimistic viewpoint. We feel that =intensive treatment has been of the greatest value in a number of cases of paretic neurosyphilis=. Two cases are given which show the most satisfactory and brilliant results of intensive intravenous salvarsan therapy in cases diagnosed as general paresis. The first case, an excellent salesman, 46 years of age, with most aggravated mental symptoms, recovered symptomatically and all his tests were rendered negative.[138] He has now remained entirely well and economically efficient for about two years without further treatment. The other case,[139] a housewife, also with very marked symptoms suggestive in all ways of general paresis, also recovered rapidly under treatment and her tests became negative. Her remission has now lasted for nearly three years without further treatment.

At times it is not possible to get the spinal fluid tests to become negative in cases of paretic neurosyphilis under the most intensive salvarsan therapy. In spite of this, the clinical condition of the patient may improve so greatly that the patient can be considered =clinically recovered=. An illustration is given of an undertaker[140] who was brought from a condition of the greatest cachexia and mental confusion to a condition of robust appearance and mental efficiency under intravenous salvarsan therapy, in spite of the fact that his tests were very slightly if at all reduced in intensity. He has been able to resume his former occupation and his former life with great satisfaction to himself and his family.

Improvement in paretic neurosyphilis under treatment is not to be expected very early. =Two or three months of active treatment= may elapse before one sees signs of improvement. Indeed, as illustrated by our case of the shipping clerk, this improvement may begin to make its appearance only after more than four months of intensive treatment consisting of two injections of salvarsan per week.[141] In spite of the long delay in this case, complete clinical recovery occurred and the tests became almost negative at the end of a year of treatment.

It is not only in the central nervous system that the syphilitic process may resist the most intensive treatment. In the case of the speculator, a victim of paretic neurosyphilis, which we cite, a perennially recurrent iritis appeared after several months of the most intensive salvarsan treatment which was apparently sufficient to reduce the symptoms of the paretic neurosyphilis,[142] but not of non-neural syphilis.

We give the case of a charwoman having the diagnosis of paretic neurosyphilis, who, under intensive treatment, made a symptomatic recovery. The interesting point in her findings is that all the tests in the spinal fluid became negative except the gold sol reaction which remained of the “paretic” type.[143] There is no general rule as to the reaction of the spinal fluid tests under treatment. At times one test is the first to disappear under treatment; again it is another. We have seen many cases in which the gold sol was the first test to become negative and others, as the case given, in which it is the last to show any change. As in our undertaker, symptomatic clinical improvement may be practically complete without any change in the spinal fluid tests.

One must remember that it is the condition of the patient that is of first importance; not so much the laboratory tests. Having shown the clinical recoveries with the tests remaining positive, we now have to report two cases in which there was =improvement= as shown =by the tests but no clinical improvement=. The first patient, a bank teller[144] of 39 years, with a diagnosis of paretic neurosyphilis, received intensive intravenous salvarsan for several months. Under this treatment all the tests became negative except the gold sol which remained of the paretic type. In spite of this, there was not the slightest improvement in his mental condition.

The second case, a young man of 29 years in whom the symptoms of neurosyphilis had recently appeared, under treatment showed a marked diminution in the intensity of the spinal fluid tests, notwithstanding which the patient became more and more demented and died after a series of convulsions.[145]

Of course, good results indicated above in some of our cases of paretic neurosyphilis are not to be expected in every case no matter how intensive the treatment. We give a case of paretic neurosyphilis in which the most intensive intravenous salvarsan therapy gave no satisfactory results. This was followed by several intraventricular injections of salvarsanized serum. The results of this combined treatment, however, were still not satisfactory, and the patient died.[146]

In order to emphasize as strongly as possible what we believe is a great =advantage of systematic intensive treatment= for neurosyphilis, we offer two cases in different time periods of neurosyphilis. The first is a printer with the symptoms of diffuse neurosyphilis six months after the appearance of his chancre.[147] These symptoms appeared despite three injections of salvarsan, injections of mercury and mercury by mouth. Under intensive treatment (meaning injections of salvarsan twice a week and continued injections of mercury), complete recovery occurred in a few weeks.

The second case is that of a waiter with signs and symptoms of neurosyphilis in whom the diagnosis lay between the diffuse and paretic forms.[148] This patient developed his symptoms in spite of continuous antisyphilitic treatment during the six years since his infection. This treatment had been comparatively mild, consisting in great part of mercury by mouth. However, he had had courses of injections of mercury and several injections of salvarsan. Under a systematic course of intravenous injections of salvarsan twice a week for a number of months, all symptoms disappeared and the spinal fluid tests became negative as well as the W. R. in the blood serum.

A final case is offered which indicates that antisyphilitic treatment may occasionally be of service in improving the mentality of a FEEBLEMINDED CONGENITAL SYPHILITIC.[149]

No attempt has been made in this section to give a per cent evaluation of the results of treatment in any one group of neurosyphilis. Two charts (charts 25 and 26), however, are appended which give an indication of some of our results. It seems to us, however, that it is too early to make any definite statements as to how far treatment will take us in the groups of neurosyphilis. We do feel decidedly, however, that many patients, in whatever group of neurosyphilis the diagnosis may place them, will respond to intensive systematic antisyphilitic treatment. =It is unfair to give an entirely grave prognosis in any case of neurosyphilis until the effect of treatment has been tried.=

In a separate section, entitled NEUROSYPHILIS AND THE WAR, we have presented fourteen cases selected from British, French and German writers in the war literature of 1914–16. Most of these cases were naturally somewhat inadequately reported under the critical conditions of literature made in the war. We present the cases for what they are worth: at all events they draw attention to the extraordinary interest of the neurosyphilis problem in relation to the war.

Such cases as A, one of tabes dorsalis apparently developing paresis by a process akin to shell-shock, is of value in the interpretation of the development of paresis in civil life. By “shell-shock” we commonly refer to a condition in which there is no actual traumatic injury of the brain. The hypothesis must be then that the explosion in some way indirectly caused an alteration of living conditions of the spirochetes, permitting the development of paresis.

Case B similarly seems to be a case in which a latent syphilis has turned shell-shock into tabes dorsalis.

Cases C, D, E bring up the question of aggravation of neurosyphilis _by_ service and _on_ service, respectively.

Case F likewise shows how, in the determination of amount of pension, the probable duration of the neurosyphilitic process is important.

Case G seems to show that war stress alone, without the emotional or physical effects of shell-shock, may kindle a latent syphilis into paretic neurosyphilis.

Case H similarly suggests that the “gassing” process may effect the same result.

Case I seems to show that the neuropathically tainted person may have latent epilepsy brought out through syphilis, the syphilis in this case having been acquired during the first summer of the war.

Case J was an interesting case of a syphilitic who, after the stress of the Battle of Dixmude, became an epileptic.

Syphilitic root-sciatica was developed in Case K at work in the war zone.

Case L is one of a civilian who apparently would not have developed paresis at precisely the moment when he did, if he had not been discharged as a German Jew from his long-held bank position in London.

Two cases, M and N, are cases of shell-shock, non-syphilitic; yet the picture of paresis in the one case and of tabes in the other was for a long time almost convincing to the examiners. They are better termed cases of pseudoparesis and pseudotabes, using the prefix “pseudo”, as usual, to signify a non-syphilitic imitation of the disease in question.

To sum up in the most general way the lessons of this book, we may emphasize again (1) _the unity-in-variety of the phenomena of neurosyphilis_, (2) _the value of a hopeful approach to the therapy of all cases of neurosyphilis_, _even the paretic form_, and (3) _the value of applying syphilis tests to every case of neurosis or psychosis_.

(1) RE _unity-in-variety of neurosyphilitic phenomena_.

The unity of these phenomena is confirmed, theoretically, by the common factor of spirochetosis: practically, by the Wassermann reaction, positive in serum or spinal fluid! Almost at this point the unity of phenomena ceases. Neither chronicity, nor evidence of mononuclear cell deposits, nor evidence of serious structural damage to the nervous system, nor presence of other positive tests than the W. R.,[150] nor existence of mental or nervous symptoms or signs, is a common feature of neurosyphilis. Sometimes the nervous system appears to harbor spirochetes in the most cordial manner as guest-friends (_paresis sine paresi_.) Again, perhaps as an expression of elaborate processes of immunity, the spirochetes take effect in relatively huge gummata. Sometimes the neurosyphilitic process rises as if by a regular process of siege from spinal nerve-root to spinal nerve-root (tabes dorsalis and diffuse neurosyphilis). Again, the nervous system is taken by storm, as it were (disseminated encephalitis). Very frequently the neurosyphilis is simply an indirect effect of blood-vessel disease, and huge masses of tissue are scooped out in necrosis with dependent secondary degenerations; and later the extinct lesions of vascular origin may or may not betray evidence of their syphilitic origin. Sometimes diffuse processes run on, apparently, with perfect fatalism to a mortal issue in a few years both with and without treatment. Again treatment appears to accomplish much (see fuller discussion under 2). The laws governing the preference of processes to lodge in membranes, vessels, and parenchyma, and in all combinations of these, have not been worked out. Hardly a case of neurosyphilis, properly studied ante mortem and post mortem, but would throw important light on our medical approach to one of the great problems of civilization, the problem of syphilis as a whole.

(2) RE _value of a hopeful approach to the therapy of neurosyphilis_.

The prognosis of neurosyphilis is not worse than that of the chronic diseases in general. In fact, the prognosis of neurosyphilis _quoad vitam_ is either good or dubious, certainly not bad. The surprising reversals of form which the spirochete shows in certain remissions are always to be awaited. Treatment of neurosyphilis has certainly effected amazing results, not so much by way of Ehrlich’s _therapia sterilisans magna_ as by means of systematic intensive treatment. Even paretic neurosyphilis (general paresis) seems to have been cured. Preparetic phases are theoretically hopeful. Nor is it so certain that paretic neurosyphilis will ultimately prove a perfectly distinct species of neurosyphilis. General paresis seems to us at least to be more closely related to diffuse neurosyphilis than is tabes dorsalis to diffuse neurosyphilis. In any particular case, moreover, =during a good part of the early months or years=, =it is difficult or impossible to tell the paretic from the non-paretic forms of diffuse neurosyphilis by any combination of clinical observations and tests=. In the instance of more protracted neurosyphilis, e.g., tabetic, the outlook for vascular complications is such that antisyphilitic treatment directed at prevention of these complications is scientifically warrantable, even if the tabetic process itself proves unassailable. The old distinction of syphilis and parasyphilis, so striking and apparently satisfactory when introduced by Fournier, seems to be a false distinction which should be dropped. Therapeutically, we should approach all cases of neurosyphilis without bias or nihilistic prejudgments.

(3) RE _universal applicability of syphilis tests in nervous and mental cases_.

The importance of putting every neurosis or psychosis through syphilis tests is not based alone on the frequency of neurosyphilis, though neurosyphilis is surely frequent enough. The importance of universally applying these tests is established by the experience of lingering doubts both in the physician’s mind and (nowadays increasingly) in the patient’s and friends’ minds, so long as these tests are not applied. Nor should the positive serum Wassermann reaction fail to be followed by lumbar puncture and appropriate tests. The general practitioner confronting neuroses or psychoses—and what practitioner does not?—must not expect valuable results from consultation with neurologists and psychiatrists when he does not carry to these specialists the results of at least the serum W. R. in his patient. Not only are practitioners, specialists, and patients subject to discomfiture on the eventual and delayed proof of syphilis or neurosyphilis, but valuable time has been lost to treatment. How often the physician of yore (and really not so long since) had to be regarded as an eccentric virtuoso if he tested urine as routine! Well, for routine use in nervous and mental diseases, the Wassermann serum reaction is at least as important as urinalysis. Nor would we cease our homily with the general practitioner. We know neurologists and psychiatrists who use the Wassermann test _only when it is likely to be positive_! But they are dying out.