III. PUZZLES AND ERRORS IN THE DIAGNOSIS OF NEUROSYPHILIS (continued 2)

The meninges show infiltration and destructive and proliferative changes of the blood vessels. Condensed extracts from Dr. Barrett’s full report follow:

There were local variations in the severity of the meningitis. The sulci showed the most marked infiltration. The slighter degrees of exudation were made up largely of lymphocytes with a few plasma cells, occasionally large mononuclear cells, and rarely 220a polymorphonuclear leukocyte. Where the exudation was more extensive, the large mononuclear cells became more common and the polymorphonuclear leukocytes increased in number. The large mononuclear cells were often phagocytic, containing from one to six leukocytes. The exudate was always most abundant about the blood vessels. The plasma cells were always most numerous in the adventitia of the veins, here greatly outnumbering the leukocytes. The polymorphonuclear leukocytes were relatively infrequent except where there were necrotic areas, which areas were usually continuous with an infiltration of a vessel wall.

As to vascular changes, the media was not often involved, nor was the adventitia so often affected as the intima. Such lesions as appeared in the intima and adventitia were infiltrative rather than proliferative. The elastica of the blood vessels proved to show but slight changes.

A characteristic change was the endarteritis,—of a focal nature with a few large mononuclear and lymphocytic cells pushing the intima inward at the edge of a lesion. In the more marked portion of the focal process, the thickness of the intima was greatly increased by proliferation. Great numbers of large mononuclear cells could be seen between the intima and the elastica. About these cells and interlacing among the other elements of the proliferating tissue was an excess of connective tissue fibres.

The meningeal veins were more often diseased than the arteries; there was adventitial infiltration with lymphoid and plasma cells; sometimes the vein walls had become necrotic and infiltrated with polymorphonuclear leukocytes.

It will be remembered that the left Sylvian artery was grossly thickened, and microscopic section of this vessel showed a partial thrombosis.

The brain showed diffuse and focal changes. The diffuse process was one of nerve cell degeneration and proliferative changes in the neuroglia and blood vessels, and no section of the many examined proved to be free from such changes, although in the majority of instances, these diffuse changes were slight. The cortical layers showed more of these diffuse changes than did the white substance. Barrett considered that the glial cell changes were more delicate indicators of the cortical changes than the nerve cell changes. He found rod cells, satellitosis, superficial gliosis, and a large gamut of changes in the neuroglia. There were two rather characteristic nerve cell changes: a shrinkage change going on to almost complete destruction, and a type of cell swelling, also apparently proceeding to complete destruction.

4. Arteritis of pia mater.

5. Focal vascular lesions. Case 57. Seven months from infection. “Disseminated syphilitic encephalitis,” Barrett. (Photographs by Barrett.)

Paretic neurosyphilis (“general paresis”)—cerebral atrophy, without meningitis. Therapeutics cannot hope to restore lost tissue. Duration. 3 years from beginning of well marked symptoms; 6 years from beginning of obvious symptoms; 12 years from a so-called “nervous prostration.”

221Among focal changes, there were four main types: Areas of encephalitis, having the general appearance of granulation tissue, areas of simple necrosis or softening, apparently directly related to vascular changes near by, hemorrhages, and certain foci regarded as gummatous.

Save for pial infiltration and a few vascular changes, there was very little change in the medulla and spinal cord. There was a hypertrophic gliosis of the margin of the medulla and cord throughout, and a focal lesion of well marked gliosis at one point in the bulb. There were no fibre degenerations in the medulla or cord, nor were there any coarse fibre degenerations in the cortex itself except in one locus, the left gyrus rectus. This case is of peculiar value in showing to what extent lesions may proceed in a period of six to eight months after primary infection.

Of course the case is in one sense entirely atypical. The lesions were not confined to the nervous system. Aside from the maculo-papular eruption and ecchymosis of the skin, there was a diffuse hemorrhage of the inner half of the conjunctiva of the left eyeball, a small hemorrhagic focus in the mitral valve, a caseous nodule, one cm. in diameter, in the apex of the left lung whose tuberculous or syphilitic nature is left in doubt; a broad milk-colored patch of thickening of the capsule of the liver. It is to be noted that there were no gross lesions of the aorta.

222

On the classical assumption that PARETIC NEUROSYPHILIS (“general paresis”) is a fatal disease, is there a disease PSEUDOPARETIC NEUROSYPHILIS (“pseudoparesis”) which may recover or pursue a long course like that of a case of diffuse neurosyphilis (“cerebrospinal syphilis”)?

Case 58. Peter Burkhardt had been an efficient highway inspector, but in his forty-fifth year he had begun to be unable to do his work satisfactorily. His wife had become somewhat afraid of him. He had had somnolent spells in his chair and had squandered money. The mental symptoms had lasted for some six months, but had become more marked during the month preceding admission. Burkhardt would at times fail to recognize his friends.

The general physical condition of Burkhardt was very good. The pupils were irregular and reacted sluggishly both to light and to accommodation. The knee-jerks and ankle-jerks were absent. There were no other neurological disorders upon systematic examination. There was a speech defect. Mentally, little could be determined except a certain sluggishness.

History and physical examination at once suggested general paresis. The serum W. R. was doubtful, but the spinal fluid reaction was positive, as was the gold sol reaction (which was “paretic”); the globulin and albumin were greatly increased; there were 48 cells per cmm. Antisyphilitic treatment, consisting of salvarsan twice a week and potassium iodid by mouth, was followed by a rapid mental improvement. After two months, Burkhardt was discharged apparently normal, with all the blood and spinal fluid tests negative. He has been taken back into the highway service.

1. What is the proper definition of pseudoparesis? Fournier termed pseudoparesis certain cases that looked like paresis but were not syphilitic in origin. Of these cases the most characteristic group is that of alcoholic pseudoparesis. It is clear that there will be no difficulty 223in the definition of a disease pseudoparesis whose entity is presented in the adjective that precedes the term (e.g., alcoholic pseudoparesis). According to this usage, a case of pseudoparesis would be one in which the symptoms and possibly some of the signs somewhat resemble the symptoms of paresis itself but for which another etiology could be fairly established.

2. Are there any cases of syphilitic pseudoparesis? We are of the opinion that the term should be dropped. It is true that there are cases which clinically look like general paresis and exhibit the appropriate laboratory signs of general paresis but seem to differ from paresis in their course even when they receive no treatment whatever. In the present phase of doubt as to the classification of paretic and non-paretic forms of neurosyphilis, it seems to us of doubtful utility to characterize a case as pseudo simply because it differs in its course, particularly as the literature has always duly recognized that a number of cases of general paresis have had long courses and sometimes very long remissions.

There is also another group of cases that have been termed cases of pseudoparesis, namely: certain cases of neurosyphilis which clinically look like general paresis and seem to be following its classical course but are interrupted by treatment. Here again it seems to us doubtful whether the designation pseudo should be attached to this group of cases, particularly while the whole therapeutic question in the paretic group of neurosyphilis cases remains sub judice. Accordingly we are tempted to include in the group of paretic neurosyphilis cases that either get well of themselves, or get well under treatment, or pursue a very long course, or are subject to very long remissions. But we make this decision in terminology without prejudice to the therapeutic question and it is open to any critic to throw these cases into an atypical non-paretic group of neurosyphilis cases.

3. How shall we explain the absence of ataxia of case Burkhardt when knee-jerks are absent and when, therefore, we are entitled to conclude a certain degree of spinal disease? As stated in connection with case Sullivan (16), the absence of knee-jerks is not a warrant for terming a case—paresis of the tabetic form. The fact is that the lesion in paresis tends to be intraspinal, just as the higher brain lesions tend to occur within the brain substance. The meninges are relatively spared both within the cranium and within the spinal canal. 224The characteristic degeneration of posterior nerve roots which we find in tabes dorsalis is not necessarily found in general paresis even when there are somewhat extensive spinal lesions. Accordingly the absence of sensory returns by way of the posterior nerve roots which characterizes tabes dorsalis is not necessarily a phenomenon of general paresis. The mechanism by which the knee-jerks are lost depends upon histological detail. They may be lost when under tabetic conditions the posterior roots are severely diseased and when under paretic conditions only intraspinal collaterals or a small portion of fibres are affected. The whole question hinges upon where and to what degree the various reflex arcs are cut in the disease. The tabetic phenomena are, as so commonly stated, intradural; that is, the sensory or gangliospinal neurones at certain levels are affected all the way in from the points at which they pierce the dura mater. The affection of these and other neurones in general paresis is an intraspinal and parenchymatous affection.

225

Neurosyphilis; auditory hallucinations; ideas of persecution; attacks of excitement. SYPHILITIC PARANOIA (Kraepelin)?

Case 59. Bridget Curley was a case that was discharged from the Psychopathic Hospital, recovered, after 26 days in hospital. The symptoms so resembled those of alcoholic hallucinosis that the diagnosis was made despite the fact that the patient consistently denied the use of intoxicants. There was, in fact, no proof that she drank alcohol. The case was, however, not clearly one of alcoholic hallucinosis or of any other well-defined form of mental disease. A provisional diagnosis of manic-depressive psychosis, manic phase, had, in fact, also been made.

The illness had begun with depression and inactivity, Bridget’s friends accounted for these conditions on the ground that a lover had departed for Ireland. A few days after the depression began, Bridget became dizzy and refused to give a boarder his breakfast, stating that she had lost her memory and had begun to hear bells ringing and people talking. She then became greatly excited and was brought to hospital, where the prolonged baths quieted her.

It seems that Bridget had had stomach trouble and headaches at the top of her head or sometimes in her temples. Physical examination showed the left pupil to be larger than the right, a slight tremor of the lips, a slight systolic murmur at the apex, slightly irregular pulse, and moderate edema of ankles. The blood serum was negative to the W. R., but lumbar puncture was executed and the fluid showed a positive W. R.

The patient was tested by the Binet and other methods, and although 35 years of age, seemed to be by the mental tests hardly over 11 years old. She was inclined to be feverish, somewhat restive, and pugnacious; rather slow of speech, sometimes refusing to answer and grimacing. Her pugnacity was, however, easily controllable, and the excitement 226was largely at night. This excitement subsided rapidly in the course of a few days.

1. What is the diagnosis in this case? The following diagnoses and suggestions for diagnosis were made at the staff meetings:

Unclassified mania.

Manic-depressive psychosis, manic phase.

Toxic delirium.

Dementia praecox.

Bacterial infection of the brain.

Unclassified delirium.

Acute delirium.

Infectious psychosis.

Acute confusional psychosis.

Psychopathic personality by use of alcohol.

Mental deficiency with atypical mental state.

Syphilitic paranoia.

2. Is this a case of syphilitic paranoia? The so-called syphilitic paranoia of Kraepelin is a rare and uncertain type of syphilitic mental disease. Delusions and hallucinations are prominent. As a rule, the onset is stated to be slow and insidious, or at any rate there are a variety of indefinite prodromata. Jealousy is a prominent feature, sometimes attended with marked sexual excitement. Auditory hallucinations and ideas of persecution are particularly in evidence. The most striking feature in Kraepelin’s group was a sudden occurrence and equally sudden disappearance of violent excitement, with or without external cause. Thus, an excitement would be produced by a few words spoken, and immediately after, the phase of excitement would pass and the patient would become entirely friendly and accessible once more, as if nothing had happened. About half of Kraepelin’s cases showed a positive serum W. R. He does not report lumbar puncture findings, and grounds the existence of disease upon certain autopsied cases. The speech and writing disorder of paresis as well as the characteristic disorientation for time and muscular weakness of general paresis were absent in the group. It appears that most cases of the group have hitherto been placed in dementia praecox.

227

The clinical symptoms of CHRONIC ALCOHOLISM are sometimes largely identical with those of PARETIC NEUROSYPHILIS (“general paresis”): differentiation by means of the laboratory findings.

To demonstrate this proposition, the cases of Francis Murphy (60) and David Collins (61) are in point, being sharp foils to one another.

Case 60. A laboring man about 44 years of age was brought to the Psychopathic Hospital one summer day, in a stupor. This patient, Francis Murphy, had been at his regular work as axeman in the Park Service, when he suddenly fell in a heavy convulsion. He was carried to a general hospital, still in convulsions, and ether was administered to quiet the movements. The convulsions shortly ceased, but the patient’s consciousness failed to clear; hence his transfer to the Psychopathic Hospital.

Here he remained much disturbed and was placed in a room with a mattress on the floor. On this mattress he would crouch on all fours for a considerable time, looking fixedly downward as if at an object on the floor, unresponsive to questions but compliant with efforts to place him on his back. He gave the impression of daze and either disorientation or confusion.

Within twenty-four hours the patient became more tranquil and consciousness became clearer, but the patient was at a loss to bring to memory either recent or remote events. However, he replied to questions, giving some different story each time he was approached. Curiously enough, the patient seemed very contented and good-natured and would even laugh foolishly at times, saying that he felt fine and all ready to go out to work.

The general impression conveyed by Francis Murphy at once suggested the possibility of neurosyphilis. Convulsions, perhaps initial in middle age, with a post-convulsive 228stupor, followed by a partial clearing up, with persistent amnesia and a suggestion of fabrications with euphoria, bore out the suggestion.

The physical examination strengthened the impression of neurosyphilis. Well developed and nourished, florid, with a manual tremor and sweating of the palms, the patient was in general without physical symptoms. Neurologically, however, whereas the left pupil was larger than the right and reacted properly to light, the right pupil was a bit contracted, somewhat irregular, and either reacted not at all to light or very slightly so (reacting perfectly to accommodation). The knee-jerks could be obtained only with reinforcement, and several other reflexes could not be elicited (triceps, radial, ulnar, periosteal, Achilles, umbilical). Moreover, the heel-to-knee test was poorly performed; some of the common tests phrases were very poorly repeated; there was marked tremor in writing; and the paragraphia seemed to be not merely peripheral, for syllables were left out in words and ordinary words spelled incorrectly (psychographic disturbance).

We do not care here to insist that the right pupil was really an example of the Argyll-Robertson phenomenon since the slightest tinge of doubt is important if a positive diagnosis is practically equivalent to asserting syphilis. Practically, however, the right pupil was regarded as an Argyll-Robertson pupil under hospital conditions (flash-light reaction). Argyll-Robertson pupil, areflexia, speech disorder, writing disorder, memory disorder, conduct disorder, and euphoria, all with a history of convulsions, certainly warranted the tentative diagnosis of neurosyphilis.

As usual, resort was made to the W. R. in the serum and in the spinal fluid. One of the first results to come through from the laboratory was the absence of globulin, normal albumin, negative gold sol reaction, and a cell count of two cells per cmm. in the spinal fluid. Later the W. R.’s were returned negative for blood and spinal fluid.

In the meantime, an illuminating change had occurred in the patient, for two days later,—three days after the first convulsion in the park,—the patient had apparently quite recovered; his consciousness became nearly clear; he could 229remember every event up to the time of the convulsion, and his memory came back in appropriate degree for both remote and recent events.

The patient, it appeared, had for some time been drinking more and more heavily. In recent days, he had been taking five or six whiskeys and a half dozen beers daily on the average, and often much more. About ten years before, the patient narrated, there had been a convulsion at a ballgame, and this convulsion the patient himself called a “rum fit.”

Here, then, is a case of Alcoholic Pseudoparesis. Without the W. serum test and without the spinal fluid examination, it is probable that the diagnosis of general paresis might have clung to the patient for some time on account of the apparent Argyll-Robertson pupil, which had to be accepted as such on the flash-light data. In point of fact, in this case the pupil later reacted more normally to light, and the speech and writing disorders measurably cleared up.

1. Can alcohol produce the Argyll-Robertson pupil? The majority of neurologists would today answer, Yes.

2. If in the case of Francis Murphy, the W. R. in the blood had happened to be positive on account of a non-neural syphilitic infection (spinal fluid negative), would the diagnosis general paresis be warranted? Probably the diagnosis general paresis would have been made. If the patient had been lost to observation, he might well have been regarded as an atypical paretic with prodromal convulsions.

3. Would positive globulin and excess albumin in the spinal fluid alone or in association with a positive serum W. R. warrant the diagnosis general paresis or neurosyphilis? The chances are that most neurologists would advocate proceeding to treatment in any case of positive serum reaction, whether or not there was globulin or excess albumin; but the positive globulin and excess albumin would probably not warrant the diagnosis general paresis or neurosyphilis in the absence of excess cells and the characteristic gold sol reaction and W. R. in the fluid.

4. Is the case of Francis Murphy one of alcoholic epilepsy (as suggested by Murphy’s own phrase, “rum fits”)? It must be remembered that epileptics become alcoholic and that epileptic convulsions increase or become 230more severe with alcoholism. On the other hand, the literature indicates that alcoholism can produce convulsions, as can many other factors. The literature also indicates that there is a condition of epilepsy in which the convulsive tendency sets in as a result of alcoholism in a patient not previously disposed to epilepsy; it appears also that sometimes, though very rarely, the epilepsy continues after withdrawal of alcohol, and even after giving up the habit. Francis Murphy appears to have had but two spells of convulsions, both of them following heavy bouts with alcohol. There is so far, then, no warrant for calling Francis Murphy’s case one of alcoholic epilepsy.

5. Does the use of alcohol by a subject destroy the value of the W.R.? It has been held by some that alcoholism interferes with the accuracy of the W. R. This has not been our experience and for the present we are of opinion that the results have the same value in alcoholics as in non-alcoholics. The next case (Collins, 61) is one in which a positive W. R. occurred in an alcoholic. When dealing with paretic neurosyphilis it is especially true that the W. R. is disturbed very rarely, if at all, by toxins or drugs, except antisyphilitic drugs.

231

Alcoholism may cloud the diagnosis of NEUROSYPHILIS. Differentiation by laboratory tests.

Case 61. David Collins was a steamfitter of about 43 years of age, picked up at 6.45 a.m. in the midst of convulsions and talking incoherently, in a state apparently of fairly clear consciousness. On arrival at the hospital, the patient was able to tell how he had always been a hard drinker, and how during the past week of unemployment he had taken large quantities of poor whiskey,—perhaps an average of a pint a day. Collins also told how he had had delirium tremens several times, but he said the present spell was quite unlike delirium tremens. There was no disorientation or impairment of memory, and the patient did not in any wise suggest a mental case a few hours after admission.

It appears, according to Collins, that he had obtained some work the night before, and had quit work about 6.30, whereupon he stepped into a barroom, took one drink of whiskey, left the barroom, walked down the street, and suddenly lost track of the world, coming to consciousness in a carriage with two policemen, but remaining, as he said, “dopy,” inattentive, and confused. After a meal, however, the patient began to feel better and soon felt quite all right.

The physical examination was quite negative except that neurologically there was lingual and manual tremor, a speech defect, apparent only with test phrases, unsteadiness of handwriting, left knee-jerk greater than right, a left-sided Babinski reflex, and a difficulty in executing rapid successive movements (dysdiadochokinesis). This degree of neurological disorder in our experience warrants lumbar puncture as well as a serum test. The lumbar puncture shortly disclosed a positive globulin and excess albumin, and the returns from the W. R.’s were positive for both spinal fluid and blood serum. The data of the gold sol reaction were not available on account of technical difficulties. However, it appears that the diagnosis of neurosyphilis could hardly be avoided in this case.

232David Collins differs from Francis Murphy, then, in showing a positive blood and spinal fluid reaction for syphilis as well as a positive globulin and excess albumin. As above remarked, it is probable that the positive globulin and excess albumin would not warrant more than a suspicion of neurosyphilis taken by themselves.

Unfortunately, we were unable to persuade the patient to submit to treatment, and from the patient’s point of view possibly his decision, not to submit to treatment, was a good one since he has had no symptoms of any sort for a period of 18 months since his episode. However, as abundantly elsewhere demonstrated, we feel that the patient is wrong, and that the physicians are right in urging treatment.

1. Is not the convulsive episode an alcoholic phenomenon in David Collins entirely separate from the patient’s general and neurosyphilis? Possibly; however, an outbreak of neurological symptoms with spontaneous recovery is not only consistent with the diagnosis of syphilis, but somewhat characteristic of neurosyphilis. We suspect that another attack will occur in David Collins.^[12] We shall from time to time make use of the social service to suggest his going under treatment, and shall employ his record of contact with a public institution to drive in our suggestion. Still it is clear that there are numerous cases in the community that are not accessible to social service initiated from a public institution. Accordingly, educational propaganda is necessary for salvage of the middle- and upper-class victims of syphilis. It is a little unfortunate that the ethics of the private practitioner make such salvage of middle- and upper-class persons not very likely. Might it not be that an extension of state medicine to this field would incidentally increase the amount of successful private practice?

2. What may be the cause of such a convulsive episode as that of David Collins? It would appear that the convulsions of general paresis and of neurosyphilis in general often occur without gross structural lesions of the brain. It may be suggested that vascular irritation or 233parenchymal irritation by spirochetes, acting in appropriate parts of the central nervous system, can produce such convulsions.

3. What is the significance of the unilateral phenomenon in David Collins (left knee-jerk greater than right; left-sided Babinski)? The current explanation of hyperreflexia is that somehow inhibitory impulses from upper portions of the nervous system have ceased to influence the local arcs that mechanize reactions like the knee-jerk and the normal plantar reflex. The phenomena are commonly found in cases with pyramidal tract disorder, and in the case of David Collins one may suspect, therefore, that there was a central disorder affecting the right pyramidal tract above its decussation. One might suspect that the convulsions were initiated by a lesion (whether gross or microscopic in range) in the right side of the cerebrum; but whether in the white matter or in the gray matter must be left doubtful. The clearing up of all symptoms suggests either that the lesion was microscopic in range or that the phenomena were transient and functional.

4. Can the dysdiadochokinesis be used to indicate cerebellar lesion in David Collins? Possibly; but it does not appear that the difficulty in executing successive movements was unilateral. It seems impossible to bring into close topographical relation the basis for the Babinski and left-sided hyperreflexia, and the basis for the dysdiadochokinesis. Alcohol is sometimes asserted to exert an especial effect upon the cerebellum.

5. Must we suppose structural lesions, either (a) of the nature of cell losses demonstrable microscopically, or (b) of the nature of secondary degenerations demonstrable by Weigert myelin sheath methods, in the case of David Collins? It appears that we do not need to assert the existence of such lesions.

6. Could the hyperreflexia and the Babinski reaction be due to local spinal cord disease? Possibly; but the existence of other neurological symptoms (lingual and manual tremor, speech defect to test phrases, ataxic handwriting, and dysdiadochokinesis) makes it probable that there were lesions, or at any rate disordered functions, within the cranium; and there appears to be no basis for asserting local spinal cord disease.

234

Differential diagnosis between NEUROSYPHILIS and ACUTE ALCOHOLIC PSYCHOSIS.

Case 62. Joseph Buck was a chef of 60 years who came in, seeking advice because his memory was getting poor; he was unable to remember names and what he was about to do. He was tremulous and had much pain in his limbs. He had been drinking heavily for weeks,—probably ten weeks; in fact, he described himself as having had “the shakes” and as having lately seen animals and people that were unreal. He had had the shakes before and the condition had lasted for two to three days after alcohol was discontinued.

Physically, Buck was tall, well developed, although poorly nourished, with a skin suggesting alcoholism. There was a slight acne over the back and chest; there was a slight enlargement of the heart, with blood pressure, systolic, 180, diastolic, 120. There was a corneal opacity of the left eye, which the patient said was the result of syphilis following a chancre, which he had acquired at the age of 27. There was also a ptosis of the upper lid of the left eye. The right pupil was irregular and reacted to light sluggishly, and with a very small excursion. The patient was slightly deaf in both ears. The deep reflexes were all lively and equal. The tremor was most marked in finely coördinated movements. There was a slight swaying in the Romberg position but the sign could not be said to be present. The gait was unsteady. There was a marked tenderness over the nerve trunks.

So far as mental examination went, it seemed that the patient’s claim of amnesia was subjective. There was certainly no more amnesia than a slight difficulty in recalling details. The diagnosis of alcoholism with convalescence from delirium tremens would certainly seem to have been sufficient for the phenomena, and the suggestion of alcoholic neuritis only confirmed the picture. To be sure, one might expect a diminution or absence of deep reflexes; still, these reflexes may be overactive in an irritative stage of the disease.

235Naturally, however, the history of syphilis and the pupillary phenomena and ptosis, made the consideration of neurosyphilis necessary. Both serum and fluid W. R.’s proved positive; there was an excessive amount of albumin and globulin, the gold sol reaction was typically “paretic,” and there were 377 cells per cmm.

The patient improved upon a rest treatment and was given injections of mercury for his syphilis. After a few months he felt well enough to return to work, and continued at work throughout a season, receiving mercurial treatment throughout this time. A spinal fluid examination fifteen months later showed a weaker gold sol reaction, reduction in the amount of globulin and albumin, and but 26 cells to the cmm. The W. R.’s had remained positive.

1. What are the forms of syphilitic neuritis? According to Nonne, syphilitic neuritis and polyneuritis have at last acquired standing in neuropathology. The older claims depended upon findings on palpation and recovery after antisyphilitic treatment. Since the introduction of salvarsan, cases of ophthalmoplegia, facial, acoustic, and optic nerve disease, as well as neuritis of the extremities, have been reported in large numbers. These phenomena are to be regarded as neurorecidives in the modern sense of that term. The neurorecidive is not a salvarsan effect, but is an effect of the syphilitic process itself, settling in the peripheral nerves. Paresthesias are especially prominent in peripheral mono- or polyneuritis, and this point is of some value in differentiating the syphilitic peripheral neuritis from root neuritis. Root neuritis is more often characterized by neuralgic attacks. Objective hyperæsthesia of neuromuscular origin is also found in these cases, demonstrated by pressure on the nerves. The motor phenomena consist in a flaccid paresis or paralysis, especially affecting the radial, ulnar, and peroneal nerves. Nonne states that it is rare for syphilis to affect a single nerve region, and he regards cases in which a single region alone is affected as usually due to a local gummatous process.

2. What is the significance of 377 cells per cmm.? See discussion of Washington (Case 66).

236

Differential diagnosis between NEUROSYPHILIS and CHRONIC ALCOHOLISM.

Case 63. Albert Fielding, 46, was an insurance broker, who was brought to the hospital for excessive alcoholism. Indeed, he showed all the signs, both of chronic and acute intoxication, except that there was no nerve trunk tenderness. Fielding was very loquacious though his speech was rather thick. He showed tremor of hands and an alcoholic skin. Physical and neurological examination proved entirely negative.

Fielding claimed that he had had a nervous breakdown at about 36 years of age, after disappointment in love. He had the drinking habit and began to drink more and more. He had now become nervous and tremulous and had to drink in order to brace himself. After a few days, the patient began to be much better, having recovered from acute alcoholism. Mental examination now showed good memory with orientation intact. There was a certain tendency to reminiscence and to somewhat childish actions. He had attempted to stop drinking but had been unable to quit. As a matter of fact, his mother and father had been excessive drinkers and he had inherited the tendency, etc.

The diagnosis seemed to be plain. The routine W. R. upon the blood serum was negative. However, the patient had remarked during the history taking, that he had had a chancre and secondary symptoms of syphilis. Accordingly, lumbar puncture was resorted to. The fluid showed a slightly positive W. R.; the gold sol reaction was of the syphilitic type; there was a considerable increase in albumin and globulin, and there were 20 cells per cmm. The diagnosis of neurosyphilis seemed clear.

Course: The patient received six months’ treatment in a sanatorium but the symptoms remained almost as before, and the patient showed the same childishness and inability to take care of himself. Since the symptoms continued six 237months after the withdrawal of alcohol, it might well be suspected that the condition was more than a merely alcoholic one. However, in a number of purely alcoholic cases, such long-standing effects are found: even as long as six months or longer after the withdrawal of the alcohol, and one might conclude therefore that Fielding was actually a victim of alcoholic dementia. The spinal fluid after these six months (during which period antisyphilitic treatment was given) showed no change, and the prognosis was offered that the case would probably develop into one of paresis.

A year later, after six months sanatorial care and six months life in the community, the patient returned to the Psychopathic Hospital in an alcoholic condition. The lumbar puncture showed all signs negative except the W. R. which was slightly positive. The W. R. of the blood was negative.

In connection with this case, see the case of paresis sine paresi (25).

1. What is the relation of the syphilitic and alcoholic process in Robert Fielding? One does not like to break the so-called rule of parsimony in diagnosis, but it would seem that the effects in Fielding are the combined effects of syphilis and alcoholism.

238

Differential diagnosis between NEUROSYPHILIS, DIABETIC PSEUDOPARESIS and BRAIN TUMOR.

Case 64. A large and imposing person, Calvin Hall, 55, had been employed as a doorkeeper and guard, in which position he was on duty for 12 to 14 hours daily. Eventually, however, he had begun to have a good deal of pain in the legs and a few months before observation, one day, his legs gave way and he fell to the floor. There was, however, no loss of consciousness, and he was carried to a general hospital. The result of an examination there was that his family was informed that he had some nervous trouble.

Hall now began to be melancholy and wept a good deal. His appetite and sleep remained intact. He felt too weak to walk. At the end of about a year, he began to improve and again became able to do a little light work. About a month before coming to the Psychopathic Hospital, about two years after the onset of symptoms, Hall suddenly began to talk excessively, in a rambling and rather senseless way. A fortnight later, he began to suffer from insomnia and restlessness.

Some medical facts were available: It seems that at 25 years this patient had become infected with syphilis though there had never been any secondary signs. He was married four years later but there had not been any children. Moreover, for four years past, the patient had been treated for glycosuria.

Upon admission, the patient’s sensorium was clear, but his orientation was only partial. He could give a fair account of his life, but it appeared that his memory was somewhat impaired. There were auditory hallucinations (voices of relatives). He often mistook the identity of persons about him. He talked in a grandiose fashion of his great strength and especially of a God-given power to read minds. His flow of thought was rapid, rambling, circumstantial, and with traces of irrelevance. He was rather continuously busy and 239at times restive. There was a good deal of emotional agitation and apprehensiveness, and again the patient would become suspicious and tearful.

Physically, there was a discharging sinus connected with the right humerus, close to the elbow. The pupils, though equal and regular, were sluggish in reaction to light. The knee-jerks and ankle-jerks were absent. There was no Romberg sign but there was some swaying in the Romberg position. There was a moderate ataxia in walking. Glycosuria to a moderate degree was determined. There were no casts or albumin in the urine. The W. R. of the blood and of the spinal fluid was negative. The albumin of the fluid, however, was considerably increased. X-ray examination of the skull yielded a suggestion of absorption of the posterior clinoid processes of the sella turcica. The X-ray examination of the arm in the region of the sinus showed a chronic osteomyelitis, possibly syphilitic (or diabetic?).

The diagnostic problems in the case of Calvin Hall are extremely intricate. There are clinical suggestions of general paresis, not confirmed by the laboratory findings.

1. Are we dealing with a case of diabetic pseudoparesis? Is the pain in the legs of like origin, and has a neuritic process led to the absence of the knee-jerks? The Allen treatment appears to have had no beneficial result in this case.

2. Is there a tumor of the sella region, which could account for the mental symptoms and the glycosuria? The spinal fluid albumin might be regarded as consistent with a variety of psychoses, including that of brain tumor. We have to remember the definite history of infection, the sterile marriage and the possibly syphilitic osteomyelitis.

240

DIABETES AND NEUROSYPHILIS, relations?

Case 65. Donald Barrie, a man of 61, diabetic for several years, had begun to worry about the diabetes, feeling that he was about to die, and had gone so far as to make several threats of suicide. Hence he was brought to the Psychopathic Hospital for observation.

Barrie was rather well developed and nourished, although he looked far older than he was. There was a marked arcus senilis; the skin was dry and rough; the radial and other accessible vessels were markedly sclerosed; abdomen obese; right testicle very low with thickened and hard epididymis.

Neurologically there was little abnormal to discover. The pupils were irregular; both reacted fairly well to light. There was a slight tremor of the extended hands, and still less of the tongue. The voice was slightly thick and the patient stumbled somewhat on test phrases. Urine: specific gravity, 1029; sugar; no acetone; no diacetic acid. Sugar 2 to 11 grams for 24 hours on ordinary diet. It proved impossible to get the patient sugar-free, either by cutting down the carbohydrates or by using the Allen method.

Mentally, the depression with reiteration of wrong-doing and self-accusation because of the contraction of syphilis, were the striking features. There was, to be sure, a slight imperfection of memory for remote events; memory for recent events and knowledge of current events was very poor. Barrie claimed that his condition was entirely hopeless, that his memory was exceedingly bad, and that he was no longer capable of supporting his family.

1. What shall be said as to diagnosis in a man of 61 with glycosuria, depression, amnesia, sluggish pupil, slight tremor, slight speech defect, and a history of syphilis? The W. R. of the serum proved positive, and also the W. R. of the spinal fluid. The gold sol reaction of the fluid was of the syphilitic type. There were 112 cells 241per cmm., there was an excess of albumin, and a large amount of globulin. Accordingly, the diagnosis of PARETIC NEUROSYPHILIS (“general paresis”), especially in view of the laboratory findings, seems necessary.

2. What is the cause of the glycosuria? First: possibly it has no relation with the syphilis; secondly: it may possibly be due to a syphilitic involvement of the pancreas; thirdly: it is barely possible that it is due to syphilitic disease of the fourth ventricle or of the base of the brain, involving the pituitary region. Perhaps our case is too complex for analysis. At all events, the case brings up the possibility of a syphilitic glycosuria.

3. Can the diabetes in the case of Barrie be explained as syphilitic? Warthin of Ann Arbor has recently described somewhat remarkable spirochete findings in his autopsy material. The order of organic infection according to frequency is: aorta, heart, testis, adrenal, pancreas, nervous system, liver, and spleen. Warthin has called attention to the relation of pancreatitis and spirochetosis to diabetes in a recent review^[13] of findings in 41 autopsied cases from the University Hospital in Michigan. Warthin found active luetic lesions in the pancreas in 6 cases.

242

Hemianopsia in a case of neurosyphilis.

Case 66. Lawrence Washington, a colored cabman, 58 years of age, began to forget addresses given him by his fares. Moreover, he could no longer see as well as before, especially on looking toward the right side. He himself states that the visual trouble dated back as long ago as his 39th year, at which time he had a terrific pain in both temples, leading back from the eyes. Washington thought that his vision had been getting slowly but steadily worse ever since.

We got the impression that the amnesia claimed by Washington was more or less subjective and he was found to be well informed. This association of amnesia and impairment of vision naturally suggests syphilis. The patient himself stated that he had had a chancre at the age of 18.

We found the W. R. of the serum to be appropriately positive. The W. R. of the spinal fluid was also positive though weakly so. There was an excess of albumin; globulin appeared in large amount; the gold sol reaction was of the syphilitic type; there were 186 cells in the spinal fluid.

Is this case one of paresis or of some other form of cerebrospinal syphilis? Let us consider the data of the physical examination. On the whole, the patient was well preserved. There was a slight radial arteriosclerosis, but on the whole the cardiovascular system was almost negative. The blood pressure was 100 systolic, 65 diastolic. Neurologically the visual field of the left eye was somewhat limited, and there was a temporal hemianopsia of the right eye. The ophthalmoscopic examination showed a disseminated choroiditis on both sides. The right pupil failed to react to light. The left pupil reacted slowly. Both pupils reacted properly to accommodation.

The knee-jerks could be obtained only on reinforcement, and when obtained, the right was apparently more active than the left. The left Achilles was absent; the right present. There were no other abnormal reflexes.

243The motility of the facial muscles was somewhat impaired. Finger-to-finger and finger-to-nose tests were rather poorly done. The muscle sense was good; there was no swaying in Romberg position; and there was no speech defect.

We are unable to decide whether the case is one of the parenchymatous type (paretic) or of the meningovascular type of neurosyphilis. It is certainly rather unusual to find hemianopsia in a paretic.

We have been unable to get definite results from the treatment of this case, since the patient would not return for months after getting an injection or two of salvarsan, on the ground that he was improved enough and did not require further treatment.

1. What conclusion can be drawn from the 186 cells per cmm. in the spinal fluid? Ordinarily this finding would indicate an active process. Some writers have claimed that a cell count running above 100 per cmm. was an indicator of diffuse non-paretic neurosyphilis. It does not appear that this claim has been substantiated. It is remarkable that this case shows an interval of 40 years between infection and the occurrence of definite clinical symptoms. With respect to the cell count, both in untreated and in treated cases, the following conclusions from a recent article (Solomon and Koefod)^[14] are in point:

1. The number of cells found in the fluid of untreated cases offers no definite information of prognostic value.

2. One is not justified in drawing any conclusions as to whether the case is cerebrospinal syphilis or general paresis, nor the time the process has been active, nor the severity of it, from the cell count.

3. The cell count may vary greatly from month to month, or when the interval is but several days, while at other times it may remain very nearly the same after an interval of months.

4. Cases showing natural remissions may show no reduction in the cell count, or other spinal fluid findings.

2445. Cases treated with salvarsan, either intraspinously or intravenously, tend to show a more or less rapid fall in the cell count. This count will, as a rule, remain low during treatment, but is likely to rise when treatment has been discontinued, but may rise during treatment after having first fallen.

6. Cases may show remissions during treatment and still have a pleocytosis.

7. Treated cases having the cell count fall to normal may at the same time become very much worse and develop more marked paralytic symptoms.

8. In general paresis the cell count in no way parallels the other spinal fluid findings.

9. In cases in which the other tests show an improvement, for instance cerebrospinal syphilis, the cell count also readily and early drops to normal. At times it may drop to normal before other spinal fluid tests become negative; again it may be last to reach normal.

10. The change in cell count seen in syphilitic disease untreated is also found in non-syphilitic diseases, as brain tumor.

11. The cell count offers nothing of prognostic importance in syphilis of the nervous system unless accompanied by improvement of the other laboratory signs.

12. The cell count is not an index to the predominance of irritative or degenerative changes.

245

Case of CEREBRAL MALARIA and SYPHILIS: simulation of PARETIC NEUROSYPHILIS (“general paresis”).

Case 67. Joseph Temple, 45, who had been a sea-going steamboat steward, was brought to the hospital in a semi-stupor. He was entirely uncoöperative, often resistive, attempting to bite the physician’s fingers, and for the most part lying curled up. He was incontinent and tube-fed. This phase, it seems, had begun the night before entrance to the hospital. Twenty-four hours later, an extraordinary change was noted. Temple became alert and attended to his wants, began to eat well, and began to behave as normally as probably he ever behaved.

He was now able to give a coherent history. It was now January. In the previous September, he had left for Mexico; he was returning when he suddenly fell to the deck, unconscious. After this fall, he had not been well, having had chills and fever. At the Marine Hospital, he had been diagnosed as suffering from malaria, and was given quinine. He had been delirious a short time in the hospital, not being able to recognize his wife, who called. He shortly improved so that his wife was able to take him home. Nevertheless, headache, gastric distress, and intermittent vomiting continued. A spell of confusion took place, two days before admission. The patient tossed about, moaned, and failed to recognize anyone. Malaria of the æstivo-autumnal type was demonstrated in the hospital. The temperature always remained at normal. He was somewhat emaciated and pale. The pupils were small, somewhat unequal, and reacted though poorly to light and distance. The tendon reflexes were lively.

The W. R. of the serum was positive, and information from the patient’s physician runs to the effect that there was a syphilitic infection some seven or eight years ago, followed by secondary symptoms, but the patient had refused to take 246any protracted treatment. The spinal fluid examination was practically negative.

Mentally, the patient was euphoric, expansive, boastful, and showed a marked emotional instability and considerable memory defect.

1. Can the diagnosis of general paresis be made in Joseph Temple? Certainly the acute confusion and the syncope are consistent enough with the diagnosis, yet the severe malaria makes it seem likely that the phenomena were due to a cerebral attack of malaria, and such occurrences are found in the æstivo-autumnal form of malaria. Yet malaria would hardly explain the euphoria, memory defect, and the pupillary findings, to say nothing of the irritability and the active tendon reflexes. Even if we regard the active tendon reflexes and the irritability as malarial, the other phenomena remain outstanding as exceedingly suspicious of paresis.

On the other hand, if we try to support forcibly the diagnosis of general paresis, we are hardly able to explain the negative findings in the spinal fluid.

In point of fact, a study of the patient’s past life revealed a story that the mental traits of euphoria, irritability, and memory defect had been characteristic of the patient for many years. In fact, there is some question whether the patient is not really to be regarded as a moron of high grade.

Upon this basis, if we regard the confusional phenomena as malarial and the persistent mental phenomena as characteristic of a moron and somewhat exaggerated by the disease, we have merely to explain the suggestive pupils. As to these, it must be remembered that though they reacted poorly to light, still they reacted somewhat, so it is not a question of explaining an Argyll-Robertson pupil, but only an impaired pupillary reaction. Of course, some workers are of the opinion that pupillary changes, perhaps even the Argyll-Robertson pupils, may occur in syphilitic cases that are not neurosyphilitic, or at all events are not victims of central neurosyphilis. Finally, we must remember that there are cases of neurosyphilis of a vascular type which yield negative spinal fluids. The case leaves many questions unanswered.

247

Can paretic and non-paretic neurosyphilis be differentiated by means of the gold sol reaction? The gold sol reaction in this case was an extremely mild one and would not at all have warranted the diagnosis GENERAL PARESIS, yet the discovery of a heavy meningeal exudate including an unusually heavy deposit of plasma cells even in the spinal pia mater will perhaps warrant us in making a final retrospective diagnosis of paretic neurosyphilis. Autopsy.

Case 68. We would like to give the full effect of our surprise at the outcome of the case of Margaret O’Brien, a school-teacher, 26 years of age. To be sure, Miss O’Brien developed symptoms at 22 or 23 which we can now explain consistently with the outcome of the case; for at that time, she began to complain of severe pain in the head, especially in the forehead and temples, and also became nervous, unable to remain quiet, and given to insomnia. She was markedly depressed at the time and would refuse to talk at times. However, only the headache in this prodromal period could be regarded as particularly suggestive of syphilis, and headache in an over-worked school-teacher is not uncommon.

In fact, the picture presented by the patient was one of catatonic dementia praecox. The patient was admitted to the hospital after a sudden onset of excitement. At first she was very restless, continually looking about and getting up and walking away from the examiner, giving the impression of understanding all questions but preserving an air of indifference. A few days later, the patient was gotten to answer more coöperatively. She remarked that the hospital was heaven although in Boston; that it was summer time (correct) and that her memory was greatly impaired. The physician was a messenger of God (delusion later corrected). The patient had not done God’s will; her breath was leaving her; God’s voice was heard from time to time, and Miss O’Brien 248had heard it for a long time. God tells her to do His will. However, as Miss O’Brien remarked, “I must think all this nonsense, turning against God.”

The patient frequently attitudinized and would remain in an apparently catatonic condition for many minutes. For the most part, she was resistive and mute and non-coöperative as to examination. From time to time, she made impulsive suicidal attempts. So far as a somewhat inadequate physical examination was concerned, nothing abnormal could be made out; in particular, the pupils reacted normally to light and were otherwise normal. The routine W. R. of the blood serum, however, returned positive, and in accordance with the policy of the Psychopathic Hospital, the patient was subjected to a lumbar puncture. The lumbar puncture yielded a positive W. R., 109 cells per cmm., a positive globulin and a considerable excess of albumin, and an exceedingly mild gold reaction—syphilitic type.

Ten days after admission, the patient had a convulsion. She never regained consciousness, continued to have convulsions for a few hours, and died, apparently from paralysis of respiration. The heart continued to beat for a short period after respiration ceased. The autopsy was consistent with the diagnosis which had been rendered after the surprising results of the W. R. in the blood and the laboratory findings in the spinal fluid had been learned. There was a generalized encephalitis with congestion of all the smaller cerebral vessels and petechial areas in the meninges and upon the cortical surfaces. We regard the case as one of syphilitic encephalitis.

The brain weighed 1265 grams, indicating a loss of 79 grams by Tigges’ formula (8 times the body length in centimetres). The pia mater was, in the gross, quite normal within the cranium; nor were any cells found in a smear from this pia mater; but the pia mater over the spinal cord was visibly edematous, and a smear from the spinal pia mater showed great numbers of lymphocytes and especially of plasma cells—a finding which was confirmed in stained section, by which a remarkable display of plasma cells was found plastered somewhat generally over the entire pia 249mater of certain segments. The brain substance was softer than normal, but displayed no differences of consistence. The stripping of the pia mater of the temporal lobes on both sides yielded the so-called “decortication” (that is, the adhesion of small bits of brain substance to the pia mater). The optic nerves were somewhat thinner than normal. No other gross lesions of the brain were found.

The dura mater, although dense and injected, was not otherwise abnormal. There was an early visible sclerosis of the middle meningeal arteries, more marked on the left side.

The cause of death, so far as the autopsy revealed it, was bronchial pneumonia. There was a diffuse nephritis.

1. Are the hallucinations in the case of O’Brien characteristic? Hallucinations are regarded as playing a minor rôle in general paresis. In fact, earlier workers sometimes denied that hallucinations occurred at all, and this denial has been made once more of late by Plaut,^[15] but Kraepelin quotes Obersteiner as observing hallucinations in 10%, and regards that figure as approximately corresponding with his own experience. Junius and Arndt are cited as finding 17% of their cases hallucinated. Auditory hallucinations are somewhat more frequent than those of vision (alcoholic psychosis must be considered). The visual hallucinations of paresis are thought by Kraepelin to be related with atrophy of the optic nerves, and he states that they occur by preference in patients having such atrophy. Hallucinations though not common are more frequent in non-paretic neurosyphilis than in paretic neurosyphilis.

2. What was the cause of death in Margaret O’Brien? The autopsy, as above stated, indicated pneumonia. In point of fact, this patient developed convulsions and ceased respiration, the heart continuing to beat for some time after respiration had ceased. It may be that the death should be counted as one of neurosyphilitic seizure.

250

Tonsillar abscess associated with neurosyphilis (Lues Maligna?).

Case 69. Frank Mason, 49 years, a rectifier of spirits, was admitted to the Psychopathic Hospital in a tremulous, mentally confused, depressed, and unhappy state. He was particularly concerned because he could not give an accurate account of his past life and because he found that he was continually contradicting himself.

Superficial examination shortly discovered the pupils to be much contracted, irregular, and non-reactive either to light or distance. Although these pupils showed more than the Argyll-Robertson phenomenon, yet the suspicion of syphilis was important.

Throat examination showed a large area of ulceration involving the whole of the right tonsil and extending even to the left side of the median line so that the whole of the faucial pillar was involved. In the midst of this ulcerative area was a mass of purulent necrotic tissue, about which the edges of the ulcer stood out sharply. There was, however, very little acute reaction about the margin of the area.

The association of pupillary changes (especially stiffness to light), what looked like tonsillar gumma, and mental disorder (including memory disturbance) heightened the impression of syphilis.

However, the remainder of the examination was not especially confirmatory of the diagnosis. The man was well developed and obese, with a slightly enlarged heart, with sounds of poor quality and the aortic second sound accentuated. The systolic blood pressure was 130; the diastolic, 90. There was no disorder of reflexes except that the arm reflexes were very lively.

After a time, a few facts concerning the patient’s life became available. Although a rectifier of spirits, Mason could not be found to have over-indulged in alcohol. It appears that some five months before his admission to the 251hospital, a wisdom tooth had been extracted. About four months before admission, the ulceration of the faucial pillar had begun, and this ulceration was immediately laid to infection from the wisdom tooth cavity. Mason then had to discontinue work and a depression followed. But the account of this depression led us to think that he was a victim more of natural sadness than psychopathic depression. There was much worry and insomnia. To meet the insomnia, large amounts of hypnotics were administered. The sequence of these hypnotics was a tremendous disturbance and continual crying out by the patient. In fact, Mason became so excited that he was removed to the Psychopathic Hospital for temporary care in the condition above mentioned.

We naturally awaited the outcome of the serum W. R. The return was negative. However, the typical position of the ulcerative lesion and the non-reacting pupils,—to say nothing of the mental symptoms and the associated tremors, with incoördination (this incoördination was non-characteristic and apparently due largely to the tremor),—led to lumbar puncture.

The spinal fluid yielded a weakly positive W. R. There was a slight positive albumin, the globulin test was slightly positive, there were 14 cells per cmm., and the gold sol reaction was of the syphilitic type. We were, then, probably entitled to conclude that syphilis was active not only in the body at large but also in the nervous system. Looking back upon the case, we considered that large doses of morphine and hyoscyamus might well have produced the marked mental confusion and possibly the tremors that characterized Mason on his arrival at the hospital.

Improvement followed after a few days of rest; the confusion disappeared and the tremors diminished; the pupils returned to their normal size and reaction; depression persisted, and the patient was very properly much concerned about the tonsillar lesion. However, further improvement did not take place under antisyphilitic treatment and patient died after several weeks from what was believed to be an embolus from the tonsil.

2521. What was the true interpretation of Frank Mason’s pupillary changes? They were probably due to the opiates, despite the fact that, taken in association with the gummatous lesion of the faucial pillar, we had regarded them as possibly syphilitic.

2. How shall the negative serum W. R. be explained? Such a reaction is consistent with the diagnosis gumma. It is, however, a little surprising that with active neurosyphilis and a relatively active non-nervous syphilitic lesion like that in this case, the serum W. R. should have been negative. Possibly a repetition of the test at various times would have shown a positive serum W. R. In any event, the fluid reaction was positive.

3. Could the tonsillar ulceration be due to dental infection? The chances are against this on account of the interval (2 months) between extraction of the wisdom tooth and the ulceration, which itself seems to be of a tertiary syphilitic nature. In point of fact, the patient admitted a syphilitic infection 21 years previously namely, at 28 years of age. At that time he took large quantities of mercury and potassium iodid by mouth.

4. Relation of the case of Frank Mason to the so-called lues maligna? The case closely resembled the cases reported by Bly. Frank Mason showed great destruction of tissue, toxemia, failure to react to antisyphilitic treatment. In both of Bly’s cases, the tonsil was the starting point of the illness; and in both cases there was a trauma of the tonsil or peri-tonsillar structures (tonsillectomy and application of caustic). In our case there not only had been extraction of a wisdom tooth, but the tonsil had been cauterized.

253

Neurosyphilis versus multiple sclerosis.

Case 70. Annie Kelly is a young Irish woman, 21 years of age, who was perfectly well until three months before her admission to the Psychopathic Hospital, when suddenly one evening she became very dizzy. This was followed by a chill and vomiting. The next day she had a sore throat but was able to be about and do her work. The dizziness, however, continued and she began to feel rather queer. Gradually it became difficult for her to walk on account of staggering.

A little later she noticed a weakness of the left side, involving face, arm, and leg; then she began to find it difficult to talk. Finally the right leg became weak, making walking practically impossible. All these symptoms grew worse and the dizziness increased. At times her vision would be blurred; there were somewhat frequent attacks of diplopia. Finally she had to take to her bed, and at last she lost control of her sphincters.

At no time did she suffer any pain. She was taken to a hospital, and after a time improved somewhat; but she was told she had a brain tumor and had better be in a large city, where she could have surgical aid if this became necessary; consequently, she was brought from Montana to Boston.

On admission to the hospital, the examination disclosed no important symptoms outside of the nervous and locomotor systems. She was unable to walk unless assisted. The pupils were large but reacted well to both light and accommodation, were equal in size, and regular. Slight nystagmus was present; there was no ptosis or strabismus; vision in the left eye was poor. The other cranial nerves showed no involvement. The tendon reflexes were all present and very lively; Babinski, Gordon, and Oppenheim signs were present on either side. The ataxia was marked, especially of the lower arms, and she had some difficulty in the alignment of the fingers. The sense of position of the limbs was 254very poor. There was some tremor, which was not of the intention type. The writing showed some incoördination. The speech showed nothing abnormal. Mental examination disclosed nothing of note objectively, but patient stated she could not think so clearly as she could formerly.

The diagnosis would seem to lie between brain tumor,—which had been suggested to the patient by her physician,—multiple sclerosis, and neurosyphilis. The numerous neurological symptoms without any definite evidence of intracranial pressure were sufficient to rule out for the moment the consideration of brain tumor. The syndrome of multiple sclerosis is not complete, but the race, age, and onset, with the increasing and decreasing intensity of symptoms are very suggestive of this diagnosis. The symptoms, of course, are all consistent with neurosyphilis also, and while the patient denied any knowledge of syphilitic involvement, the examination of the blood and spinal fluid was made. The W. R. was negative in both the blood serum and spinal fluid. Further examination of the spinal fluid showed presence of globulin and an increase in the albumin content, 43 cells per cmm. and a “paretic” type of gold sol reaction. With the negative W. R. of both blood serum and spinal fluid, and with so much in favor of Multiple Sclerosis, this diagnosis was made.

1. What is the relation of multiple sclerosis to syphilis? There is no definite relationship between multiple sclerosis and syphilis,—that is, multiple sclerosis is not a syphilitic disease; but the complete syndrome of multiple sclerosis is often given by a syphilitic involvement of the central nervous system (see case Lauder, 71).

2. Is the spinal fluid finding in this case consistent with multiple sclerosis? According to Nonne, about 19% of the cases of multiple sclerosis show globulin and pleocytosis in the spinal fluid. As a rule, the number of cells ranges between 10 and 20 per cmm. and the globulin is not present in large amounts. In this case, the amount of globulin, which was given as 2+, is only a moderate amount,—less than is usually found in cases of general paresis. There are not very many 255cases of multiple sclerosis in the literature in which a gold sol reaction has been performed, but in the majority of those tested, the reaction is reported as mild. However, cases of multiple sclerosis giving a typical paretic curve have been described by a number of observers, among whom may be mentioned Kaplan and Solomon.

3. How frequently is it necessary to make a differential diagnosis between multiple sclerosis and neurosyphilis? Before the days of the W. R. this differentiation was much more difficult than at present. But we, however, still have to face a not very rare difficulty in separating the two conditions. Syphilis is prone to cause small localized lesions in the nervous system. The changes in the patient’s condition, with improvements and regressions are equally characteristic of both diseases. How closely the symptomatology of neurosyphilis may simulate that of typical multiple sclerosis is shown in the next case (Lauder, 71). When the sclerotic area of multiple sclerosis occurs in appropriate parts of the cerebrum, symptoms of mental disturbances will occur. In its histological picture multiple sclerosis is at times highly suggestive of syphilis, even showing mononucleosis and meningitis.

256

Optic atrophy; nystagmus; spasticity; intention tremor. Diagnosis: ?

Case 71. James Lauder began to lose his eyesight at 32 years, and was shortly determined to be suffering from primary optic atrophy. In the course of a year, he had become completely blind. No mental symptoms had developed.

Physically, Lauder was in very good condition. Neurologically, there was a complete optic atrophy with paralysis of the internal rectus muscle, marked nystagmus, and absent pupillary reactions. All the tendon reflexes were exceedingly lively, though the right arm reflexes were more lively than the left, and the left leg reflexes more lively than the right. There was an ankle clonus on both sides. The abdominal and cremasteric reflexes were lively. There was a slight intention tremor. There was, however, no ataxia and no speech defect.

Diagnosis: The nystagmus, optic atrophy, and the reflex disorder suggested multiple sclerosis, although the liveliness of the superficial reflexes, especially the abdominal reflexes, was a point somewhat against any advanced degree of multiple sclerosis. It would appear that the absence of pupillary reaction to accommodation is also rather unusual in multiple sclerosis.

The serum and spinal fluid W. R.’s proved positive. There were 25 cells per cmm., albumin was in excess, and there was a positive globulin reaction.

1. What is the significance of optic atrophy and other optic changes with respect to neurosyphilis? Canavan, from our laboratory, has reported that she found that 40 of 58 unselected cases of mental disease exhibited obvious and undeniably important changes in the optic nerve. She found that optic nerve changes were even more frequent than chronic spinal cord changes as detectable by the same method (Weigert myelin 257sheath method); there were only 34 of the 58 cases which showed chronic spinal cord changes. Eighteen cases very probably syphilitic (although the clinical evidence was not in all cases supported by the W. R.) failed to show optic nerve changes in but three instances. The 15 syphilitic cases that did show optic nerve changes showed them in but one eye in three cases, in both eyes in 12 cases. Canavan incidentally demonstrated a spirochetosis in the pial sheath of the optic nerve in a case of neurosyphilis, possibly paretic.

2. What is the frequency of eye changes in neurosyphilis? Posey and Spiller (“The Eye and the Nervous System,” 1906) quote Kéraval as finding 42 instances of fundus change in 51 cases of paresis. Clifford Allbutt found 41 cases of atrophy in 53 of paresis; other authors have found far fewer. Optic atrophy sufficiently marked to cause blindness is relatively rare in paresis. Compare table of eye changes from Joffroy under Case Falvey (55).

As for optic atrophy in tabes, Posey and Spiller record statistics as so various as to be on the whole unsatisfactory. The highest percentages found appear to be those of Mott, 80%, and Gross, 88%. It is evident that the standards for measuring optic atrophy must differ very much.

258

Atypical case of neurosyphilis. Picture of Huntington’s chorea.

Case 72. Margaret Green, 28, was received at Danvers State Hospital in an excited and frightened state. She was very talkative and said that she was being bitten by snakes and serpents. She thought every one approaching her was the devil, and sprinkled what she called “holy water” about her for protection. It was clear that she was hallucinated. She heard her child crying, and she saw a woman carrying it away.

After a few weeks, Mrs. Green grew quiet and more rational except for a few spells of violence and noise; she gave the impression of a rather pleasant and agreeable, though somewhat demented, patient. Physically, beyond a tremor of fingers and tongue and lively knee-jerks and some evidence of enlargement of the heart, there was nothing to be found.

Margaret Green is still in the Danvers Hospital, being now 48 years of age. During the twenty years, she has presented,—besides the mental picture of impairment of memory—occasional spells of confusion, a variety of delusions based, at least in part, upon auditory and vivid visual hallucinations, a certain irritability and psychomotor excitement, and a picture of Huntington’s chorea. The diagnosis of Huntington’s chorea has always been in doubt by reason of the lack of any evidences of hereditary taint; it has, however, not been possible to secure a properly intensive account of her relatives.

It appears that the choreic movements were first observed—in the hospital at least—about 16 years ago. The patient has always been decidedly mixed upon dates. From internal evidence derived from her obviously in part erroneous statements, it may be that the chorea began at the age of 23. It appears that she had been often termed a victim of St. Vitus’ dance, and had had to leave her work in the mill on account of the disease. From one source of information, 259it would appear that the patient began to have what was called St. Vitus’ dance when she was 14 or 15 years of age; so far as this informant knew, no other member of the family had had the affliction.

The first movements observed in the hospital were irregular, jerking movements, more marked in the left arm but also occurring in the other extremities, as well as in the face, wherein were produced peculiar grimaces. The twitching movements would become decidedly worse during spells of irritability. Observation in the patient’s early thirties left the question in doubt whether the left pupil reacted to light or not. In 1904, when the patient was 36, both pupils failed to react to light either directly or consensually. At this time, the jerky movements continued, especially in the left hand and forearm, the tongue was tremulous, test phrases were poorly pronounced, the knee-jerks were exaggerated (especially the left), and both wrist-jerks were exaggerated. The systematic examination, however, revealed no other neurological disorder. Within a year, slight spurious ankle clonus developed on both sides; the eyes, especially the left, gave the appearance of developing cataracts. A slight consensual light reaction was demonstrable on the right side, but all light reactions were absent in the left eye.

At the age of 42, the patient was still disoriented for time, place, and persons and subject to a deep amnesia; was tidy, tranquil, and of a pleasant demeanor, but many of her muscles were in continual motion. There were chewing movements and both hands and feet were rarely still. There were no longer any spells of irritability or violence; and once when found crying on the piazza, Mrs. Green, on being asked the reason, replied that a gray cat had come and looked at her so hard it made her cry. There were other crying spells at times for equally good reasons, or for no reason.

More recently, the patient has become fairly well oriented for time and place, and has acquired a fairly good insight into her condition and a good memory for past events. She has had occasionally auditory hallucinations, as of water running. In 1914, it was reported that the pupils reacted 260to light, and the rest of the systematic neurological examination was negative except that the knee-jerks were exaggerated; and a re-examination in 1916 showed the pupils still reacted to light. At present, the patient is disoriented for time, stating that her age is about 25; she is no longer subject to auditory hallucinations; she has a marked difficulty in enunciation, emphasized by the lack of teeth and in part due to continual movements of the tongue; the movements appear to be part of a generalized chorea.

In a systematic review of the Wassermann findings in the hospital population, the blood of Margaret Green was examined and found to be positive. Lumbar puncture forthwith performed showed a positive W. R. in the fluid; there was a positive globulin and an excess of albumin; the gold sol was characteristic of paresis; there were, however, but three cells per cmm.

1. Are the choreiform movements related to the demonstrable syphilis of the nervous system? Neither the fluid W. R. nor the gold sol reaction should be regarded as necessarily an indicator of tissue loss. The fluid W. R. is commonly thought to signify merely that the fluid contains substances which are somehow due to the presence of spirochetes in some region pretty closely related with the fluid. The gold sol reaction, although well established to be characteristic of neurosyphilis, is perhaps not so strong an evidence of the existence of spirochetes in the region from which fluid constituents are derived. There is no pleocytosis. However, the positive globulin test and the excess of albumin do indicate a certain amount of destructive process somewhere in the neural tissues. Are we to suppose that these substances have been continually found during the course of this disease? This question cannot be answered with the data in hand, and we can only suspect that these positive tests for albumin and globulin are an effect of tissue destruction caused by neurosyphilis. It must be admitted that the argument here is a little tenuous. The lesson is plain: that in the present stage of our knowledge the W. R. should not be omitted even in cases which present a fairly convincing picture of some well-known entity. Thus, a disease, which looks like Huntington’s chorea, 261as well as a disease suggestive of multiple sclerosis, requires investigation by the methods of the syphilographer.

2. How shall we explain the changes in pupillary reaction in this case? They cannot yet be explained. A few observers have reported changes in pupillary reflexes in the direction of normality. In our experience such changes have not been noted. It cannot be too strongly emphasized that it is very easy to make errors in judging pupillary reaction if care is not used. For instance, if the patient is accommodating for near vision, light will probably not cause contraction. A frequent cause of error in testing the light reflex arises from using a weak electric light. An electric flash-light is much less efficient than daylight. Probably the most satisfactory method is to take the patient to a window, ask him to look at a distant object, shade the eye with the hand, remove hand, and observe.

3. What is the chief triad of symptoms in Huntington’s chorea? (1) Choreiform movements associated with (2) progressive mental enfeeblement, (3) occurring in a patient whose family history shows a similar condition in a preceding generation.

262

Differential diagnosis between NEUROSYPHILIS and SENILE ARTERIOSCLEROTIC PSYCHOSIS.

Case 73. Marcus Chatterton was a retired sea captain, 75 years of age. At the age of 71, he had had a seizure with a slight right hemiplegia and inability to talk. He had been slightly confused for a short time but had rapidly recovered. During the intervening four years, there had been three similar attacks, and the last one had caused him to come to the hospital. He was, in fact, confused upon admission but had become perfectly clear by the next day. There was a considerable memory defect, which the patient himself did not entirely appreciate. Possibly his judgment had been deteriorating slightly. He had been irritable of late and sometimes sleepless.

Physical examination showed a rather well preserved man with but slight senile changes. The pupils were equal and reacted readily to light and accommodation. There was no sensory disorder and no disturbance of coördination. There were no tremors. The systolic blood pressure was 205, the diastolic 135. The arteries were sclerotic upon palpation. A sufficient diagnosis would have seemed to be arteriosclerosis, and the hypothesis of syphilis would hardly have been raised off-hand by most practitioners. The W. R. of the serum was negative. What led to lumbar puncture in this case was the fact that the sea captain’s wife had died 15 years before of general paresis. The lumbar puncture was rewarding since the W. R. was positive. There was an increase of albumin and globulin, a “paretic” type of gold sol reaction, and 56 cells per cmm.

Accordingly, we must regard the condition as one of neurosyphilis. Perhaps the arteriosclerosis was of syphilitic origin. If this is a case of general paresis as we suppose, it is one of very long-standing syphilis.

2631. Do delusions of grandeur in the senile period suggest syphilis? Not necessarily; it appears that there is a small group of senile cases which might be called cases of senile pseudoparesis in which extravagant delusions of grandeur are entertained, and in which frontal atrophy is found although entirely without evidence of chronic inflammation. It has not been proved that these cases are of syphilitic origin. It is suggestive that the site of the most extensive lesion is precisely the site of the most extensive lesion classically found in paretic neurosyphilis, viz., in the frontal regions.

2. Is neurosyphilis frequently found in both mates? It can hardly be said that this is a usual finding. However, it is far from rare, and it occurs frequently enough to be used in support of the theory that there is a special strain of spirochete that has a predilection for nervous tissue. It must be remembered, however, that the wives of syphilitics are frequently infected without being aware of it. In such cases they receive no treatment and consequently have a larger chance of developing neurosyphilis. It is a good rule to consider the mate of every syphilitic a candidate for neurosyphilis.

264

An atypical case of recurrent dazed states resembling HYSTERICAL FUGUES. Probably an instance of NEUROSYPHILIS.

Case 74. Abel Bachmann, a man of 40 years, remains doubtful and perhaps belongs to the still unresolved group of mental cases due to syphilis that cannot be placed in any of the well-known categories. Bachmann had been found by the police, working in front of a cowbarn without the consent or even the knowledge of the owner. Bachmann had, in fact, spent the night in the cowbarn and was working with the idea of paying for his night’s lodgings. The situation struck the police as so peculiar, and Bachmann was so confused and irresponsive, that he was brought to the Psychopathic Hospital. The afternoon of his admission, however, he entirely cleared up and was able to give a good account of himself.

His story was that he had been worrying a good deal about a divorce suit, and the morning of his episode he had awakened with peculiar feelings. He walked from Boston to Cambridge, feeling that he was in a strange city. He recognized the places he passed, yet they all seemed to be changed. Upon reaching Harvard Square, he determined to return to Boston and walked and walked, failing to reach Boston. All day he had eaten nothing; when night fell he stole into a field and dug out radishes. A postman stopped and said, “Hello, Bill,” which awakened him as by an electric shock. A barn presented itself, in which he spent the night. In the morning, the barn looked different. In fact, his entire surroundings appeared mysterious. As he felt like working, he went to work in front of the barn.

It seems that in his life there had been two other episodes of a similar nature; in fact, Bachmann had been in a state hospital for six weeks after the first episode. The first episode had lasted a few days only, and followed worry when he learned that the girl with whom he was in love was married. 265The second attack followed the death of his mother, whereupon he was taken to a state hospital although the total duration of symptoms was only three days. Bachmann had had a chancre or some other form of genital disease at 26, and had at that time been treated with mercury.

Except for irregular and absolutely rigid pupils, reacting neither to light nor to accommodation, Bachmann showed no physical and especially no neurological disease whatever. Moreover, the W. R. in the blood serum was negative.

As to diagnosis, one might consider hysteria, of which, however, there are no visible stigmata. It would not appear that brain tumor would be likely to have lasted so long as eight or nine years, even if we should attempt to make the hypothesis of tumor cover both the non-reacting pupils and the episodes. Bachmann was non-alcoholic, and there was no sign of any other form of intoxication. The spinal fluid showed a negative gold sol reaction, there were no cells in the fluid, there was no globulin; albumin was normal. However, the W. R. was strongly positive.

The situation, then, in this case is that we have somewhat peculiar psychopathic episodes, pupils rigid to light and accommodation, a positive W. R. in the spinal fluid, and extremely little else to permit a diagnosis. We are ignorant as to the course and pathology of such cases. However, we cannot resist the temptation of the diagnosis of neurosyphilis, although further classification is not ventured.

1. What is the significance of stiff pupil as an isolated symptom? Nonne finds that in the end, after years of observation, the Argyll-Robertson pupil turns out to be an advance courier of other more functionally serious signs and symptoms of neurosyphilis. We can confirm this experience and regard it as an established clinical proposition that the Argyll-Robertson pupil cannot be neglected. In this connection, refer to the case of alcoholic pseudoparesis (Murphy, 60), and also to the case of pineal tumor (Donald Falvey, 35). Enthusiastic reports have occasionally been made upon apparent restoration of the true syphilitic Argyll-Robertson pupil to normal light reaction. The difficulties in rendering the symptomatic diagnosis of 266Argyll-Robertson pupil in a given case are so great, and the chances of complication so numerous, that we are inclined to attach little significance at present to these claims.

It may not be amiss to mention a somewhat humorous incident familiar to some local neurologists. A case was reported by the interne for a number of months as a victim of a pupil stiff to light and accommodation, and the entirely adequate cause of this phenomenon was actually only discovered at autopsy by the triumphant medical examiner, who demonstrated that the patient in question was possessed of a glass eye.

267

TABETIC NEUROSYPHILIS (“tabes dorsalis”) versus PERNICIOUS ANEMIA with spinal symptoms.

Case 75. Mrs. Brown was a woman of 56, who for the past eight or ten years had been complaining of trouble in her legs. As she described it, at times her legs were so weak she could hardly stand; at other times there was considerable pain and numbness. She has always been considered “high strung”; that is, she had a very bad temper and lost control of herself almost entirely when she became excited. Her legs had been growing progressively worse, and for about a month prior to admission she had been unable to stand or walk. She had also lost control of her bladder. On account of her temper, it had been almost impossible to nurse her; no nurse would stay with her because of her scolding and fault-finding. Recently, she had been having fits of the blues.

Her husband, who was seen before Mrs. Brown, was an old gentleman, over 70, who was chiefly remarkable from the fact that he had unequal, irregular pupils, which reacted neither to light nor accommodation; there was also a speech defect.

The patient herself proved to be extremely irritable, as had been stated,—so much so that at times it seemed almost impossible to do anything for her. She was very querulous, constantly complaining, and not satisfied with anything that was done. Aside from this, her mental examination proved to be entirely negative; that is, there were no psychotic symptoms.

The systematic physical examination gave the following significant findings: blood pressure, 160 systolic, 90 diastolic; no evidences, however, of peripheral arteriosclerosis. Patient was unable to walk or stand, and had no control over her bladder. The knee-jerks and ankle-jerks absent on both sides; ataxia in the leg movements; loss of sense of localization, 268with no tenderness over the nerve trunks; no atrophy, paralyses, or muscular asymmetry of the parts. The vibratory sense was maintained. Subjectively, the patient thought that the vibratory sense differed in the legs from that in the arms. Localization, touch, pain, heat, and cold responded to correctly. The arms showed nothing abnormal; there was no incoördination, dysmetria, or dysdiadochokinesis. Her pupils were equal, regular, and both reacted normally to light and accommodation.

Diagnosis: The first consideration in the case is naturally tabes dorsalis, especially when one considers that the husband had signs which suggested syphilis of the nervous system. The rapid onset of the acute symptoms in this case, and the absence of the characteristic signs of pain were at least atypical for this diagnosis, as was the absence of any pupillary signs. Further, the W. R. was negative in the blood and spinal fluid; there were no definite signs of inflammatory reaction as shown by the other spinal fluid tests. These findings made a diagnosis of tabes entirely improbable. After tabes, the most frequent cause of the symptoms above enumerated is perhaps to be found in pernicious anemia. Examination of the blood showed that the patient had 2,500,000 erythrocytes per cmm. The hemoglobin by Tallquist scale was 80%. The smear was practically negative; no blasts could be seen. Although this picture is not a typical one for pernicious anemia, at least it is significant in the low number of red cells to be found, and as no causes for anemia were to be found, it seemed probable that we were dealing with a primary anemia. The diagnosis in the case, therefore, is spinal sclerosis of primary anemia. The mental picture was not uncharacteristic of Pernicious Anemia.

1. Could the diagnosis be rendered in this case without a lumbar puncture? In the first place, the emaciation is not entirely characteristic. The pupils react normally to light. Probably such a case might well have been regarded as one of tabes dorsalis in former days, or even at the present time, if a lumbar puncture had not been resorted to.

2692. Could this case possibly have been one of tabes dorsalis with negative spinal findings? Such cases have been reported frequently, but, unlike the present case, are apt to be of long-standing and non-progressive, in which the active inflammation is no longer present. The negative findings would not be consistent with tabes, in which the symptoms are of short duration and of sudden onset.

3. If the serum W. R. had been positive would the diagnosis have been neurosyphilis? We are loath to make the diagnosis of spinal syphilis when the spinal fluid is normal. Syphilis may produce a marked anemia, however, and thus produce symptoms such as shown by Mrs. Brown. It is even possible that such is the explanation of this case, taking into consideration the suggestive findings in the husband. However, there is insufficient evidence to make such an hypothesis rock firm, and we do not more than suggest it.

270

Atypical case of CONGENITAL NEUROSYPHILIS—peculiar mental state.

Case 76. James Seabrook, 20 years of age, obviously showed a number of signs of congenital syphilis. The physical examination disclosed an old scar and indentation in the right mastoid region, another on the right side of the neck, another on the posterior surface of the right forearm, and two on the outer surface of the right upper arm. The lesions were about the size of half a dollar. There was a saddle-shaped nose and a perforation of the palate and uvula; there were palpable cervical and axillary glands, small but numerous. There was a dulness in the region of the right scapula, and slight dulness on both sides behind. There were loud whispering and piping râles and bronchial breathing throughout the chest, more marked on the left; there was much coughing, and the sputum was at times blood-stained. The pupils were irregular but reacted perfectly. The left knee-jerk was slightly more active than the right. The W. R. in blood and fluid was negative; the gold sol, globulin and albumin tests were negative. There were, however, 56 cells per cmm. in the fluid.

We learned that the patient had had several spells of great excitement, with pounding on the door and a desire to fight bystanders. There were spells of headache and vertigo. Mentally the tests showed him to be subnormal.

The diagnosis Of Congenital Syphilis seems established; possibly the pulmonary condition is syphilitic. The mental subnormality as well as the abnormal traits and episodes are probably to be accounted for on the basis of syphilitic involvement of the brain.

1. Are the headache and vertigo connected with syphilis? This is perhaps suggested by the pleocytosis in the spinal fluid.

2. How shall we explain the negative W. R.? This patient had received, shortly before his entrance to the hospital, 271salvarsan and mercury. Possibly the drug treatment has little or nothing to do with the negative W. R.’s since they not infrequently grow weaker as congenital syphilitics grow older.

3. What is the explanation of the spells of excitement? Compare the spells of excitement in a form of neurosyphilis described by Kraepelin, namely: syphilitic paranoia, discussed in the case of Bridget Collins (59).

4. Is treatment indicated considering the W. R.’s to be negative in blood and fluid? Despite the negative W. R.’s in this case treatment is strongly indicated on account of the pleocytosis. This would seem to indicate that there is an active inflammatory process in the cerebrospinal axis, and it is more than probable that this process is syphilitic. How much real improvement of the symptoms would result from antisyphilitic treatment it is impossible to prophesy. Every case is a special problem, and this case is very unusual in showing a pleocytosis in the absence of other indications of syphilitic nervous disease, viz., globulin, albumin and W. R.’s.

272

CONGENITAL NEUROSYPHILIS resembling an undifferentiated case of FEEBLEMINDEDNESS—actually PARETIC.

Case 77. John Friedreich, a 7–year old boy, was brought to the Psychopathic Hospital by agents of a charitable society, who found him a neglected child and quite evidently a subnormal one.

The dominance of syphilis in the situation was clear. The boy’s father had died but a few months before of syphilitic heart disease, from which he is said to have suffered for five years. The boy’s mother (the parents were first cousins) had also been treated for syphilis and was excessively alcoholic. The first child of this union—a girl—had died at 6 years, of a disease diagnosticated spinal meningitis. The history indicates that syphilis was acquired after the birth of this first child; but in any event it is possible that the meningitic condition of which the first child had died was syphilitic. The second pregnancy terminated in a stillbirth; the third issued in a girl, who died two weeks after birth of what was termed “inward convulsions.” The fourth pregnancy resulted in a miscarriage; the fifth in our patient, John Friedreich. The sixth pregnancy resulted in a girl, now 5 years of age, who is apparently normal. (Her W. R. was negative and she shows no stigmata of syphilis.)

The patient, John Friedreich, at some very early age had a rash on his body diagnosticated as syphilis. He also had many seizures called fainting spells. Ever since birth he had been taking mercury pills. He had not learned to talk until his third year, and was able then to say only a few disconnected words. In fact, John has never been able to talk in complete sentences, mumbling much that is quite unintelligible. However, he walked at 15 months in a normal fashion and nothing peculiar in his gait was noted until he was 5 years old, when he began walking on his toes, particularly those of his left foot. Shortly thereafter, the seemingly inevitable trauma appeared; John fell out of a window and severely injured his left leg, whereupon the peculiarity of toe-walking became more pronounced and associated with a limp.

Juvenile paresis. 7 years.

273The patient strikes one physically as having the development of a child of about five years (actual age, 7). There are a few lymph nodes palpable in the anterior triangles of the neck. The dilated and slightly unequal pupils react neither to light nor accommodation. There is practically complete deafness; loud sounds are not at all noticed.

Withal, the child in a general way presents a somewhat attractive appearance, being very playful and mischievous, lying about on the floor and playing with whatever comes to hand, talking to himself or making a few indistinct remarks to the bystanders. He walks awkwardly, on the toes of the left foot. He pays little or no attention to his toilet and needs to be dressed and cared for in all ways. He is quick-tempered and at times very difficult to manage.

There was, of course, little doubt of the diagnosis of Congenital Syphilis and of Feeblemindedness. The W. R. was positive both in the blood and in the spinal fluid. The gold sol reaction of the fluid was of the “paretic” type; there were 44 cells per cmm. and there was a large excess of albumin and much globulin.

As to prognosis, there is doubt.

1. Is, or is not, this a case of juvenile paresis?

2. Is it, perhaps, a relatively permanent case of feeblemindedness due to congenital syphilis? On the whole, on account of the spinal fluid symptoms, we should be inclined to give the case a relatively poor prognosis, namely, of death in a few years. However, we may perchance be later surprised to learn that the patient has lived on, at least into early adult age.

Note: Mercury tablets in some cases of congenital syphilis do not seem effective. John Friedreich was treated most intensively by syphilographers from birth.

Dr. W. E. Fernald in a personal communication stated that syphilitic cases of feeblemindedness are rather those of the imbecile and idiot groups than of the higher levels. This statement emphasizes again that the true hereditary 274cases of feeblemindedness are rather those of the higher group, whereas the cases in which special causes have operated in the uterus or in early life eventuate in idiocy and imbecility. However, such a case as that of Friedreich shows that now and then a case of feeblemindedness without evidence of neurological disorder and looking in almost all respects like an hereditary case may be at times produced by syphilis.