Part 35

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]