Part 20

5. 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.

=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 any 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.

=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 had 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 mater 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.

=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 hospital, 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.

1. 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.

=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 very 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 cases 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.

=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.