Part 11

=Case 26.= John Morrill, 49, an operative in a mill town in Essex County, Mass., was described as a “Saturday night and Sunday drinker,” with a history of very serious long sprees at the age of 43. It seems that he had had what was called “sciatica” at 35, and was treated in hospital for seven weeks at that time. The nature of this sciatica is in doubt, but there was a history of syphilitic infection at 36 years (scar of glans).

Morrill had been married twice, and two of the children were dead; one daughter was described as “very nervous,” but there were four children under ten years of age, all regarded as perfectly healthy.

Morrill had been a mill operative of average capacity, was industrious, and had supported his family despite alcoholism. The syphilis had been treated with reasonable thoroughness.

Aside from alcoholism, there had been no symptoms up to two months before admission to Danvers Hospital. Then there had been insomnia, fatigue, agitation, eruption on foot, loss of ten pounds in weight, hypochondriacal fears, apprehensiveness for the future of the children, incoherent talk; and just before admission, his talk was described as foolish. He had taken to running away and hiding in bushes by a pond and in the cellars of other people’s houses.

The patient was of medium height and weight, with thin grayish hair and grayish irides; musculature was slender. The face was blank in expression, the teeth poorly preserved with atrophy of gums, the tongue coated, and the breath foul. There was a gummy secretion of the eyelids, an area of brownish branny eruption over both clavicles, a number of depressed scars over the limbs and back, and another area of scaly eruption on the right heel and the sole of the foot. The heart area was increased, and the sounds were faint at the base, with the first sound accentuated at the apex. The urine showed a trace of albumin.

=Neurologically=, the Romberg position was maintained with a general tremor and fluttering of the eyelids. In complicated movements, the patient was slightly ataxic. The pupils were irregular, the left being much larger than the right. There were no light reactions to be obtained in window light. The reaction to accommodation was present, though slight. Vision was poor, ¼-inch capitals could not be read by left eye at reading distance. The knee-jerks were diminished equally; the Achilles jerks were absent; the other reflexes were normal. Upon the sensory side, the patient gave a history of pains in the legs at irregular intervals for several years. These pains he described as of a darting character. There was little or no sensory disorder, although the outer surface of the right leg required a deeper pressure to elicit sensation. There were no disorders of muscle sense.

If Morrill was to be trusted, he had been born in Ireland, and had come to the United States at the age of 17. He married at 18; there had been seven pregnancies by the first wife, with one stillborn child; one child had died at five weeks. The four children by the second wife were healthy. The first signs of neuritis had occurred at 45 and had received the diagnosis neuritis, although no connection between the neuritis and the syphilis had been noted.

The patient entered the hospital July 26, 1904, and was discharged, improved, January 5, 1905. He returned a little more than a year later, January 15, 1906, and died March 21, 1906. The total duration of the disease from the onset of mental symptoms may therefore be stated as somewhat under two years. When the patient appeared at the hospital the second time, he showed a positive Romberg sign, an unsteady gait, an ataxia that still was moderate, and somewhat more marked tremors, involving fingers, tongue, and face. He was now unable to read ½-inch type with the left eye. The knee-jerks, formerly diminished, were both exaggerated, the left slightly more so. The Achilles reaction, not obtained formerly, now appeared on the right side. The pupils reacted as before. The sensory loss had become more marked, since sharp and dull points could hardly be distinguished. Deep pinpricks were not felt in the leg, and heat could not be told from cold.

The speech in 1904 had been somewhat defective (“truly rural” rendered as “tooly lualal,” “sifted soft thistles” as “thoft thsistles”), and there had been little further development of the speech defect. The handwriting had lost appreciably in legibility and had become much more tremulous. During the first period of hospital observation Morrill had what might possibly have been visual hallucinations, but it was impossible to tell whether his story of seeing his wife and children trying to get in through the window was hallucinatory or a matter of fabrication. Memory was decidedly imperfect and few details of recent events could be produced. The association of ideas was almost a so-called “flight” of apprehensive, fearful ideas, loosely connected, incoherently expressed, and dealing chiefly with his work and his children. Judgment was imperfect; the height of the room was estimated as 24 feet, but the height and weight of persons were estimated with fair accuracy, and also the length of small objects, whose lengths were doubtless remembered rather than estimated. The estimate of time elapsing during a medical examination was accurate, but the estimate of longer durations involving over-night memories was hopelessly imperfect. Emotionally, there was a dulling of sensibility, an appearance of suspicion and apprehensiveness; the patient fancied himself to be in a hopeless condition as a result of syphilis, but at the same time accompanied his statement of his hopelessness with laughter. A sample of his hypochondriacal ideas: “I am all gone; I am good for nothing; I am all gone now; I can’t drink now; can’t write or talk at all; worse than when you saw me first; nothing in my inside; all wrong through me again; I aint got no swallow now; I can’t die even; my heart aint much good; I can’t hear it beat; I don’t think it flutters; no life in these hands; they are all cold and dead” (pointing to his arms and moving them about). During such a portrayal the patient laughed in a silly way.

During the second hospital stay, Morrill was at first restless, sleepless, profane, imperfectly oriented for time, possibly for place, and also for the attendants. A few weeks later he became stuporous and confused, and his feebleness and physical exhaustion were finally ended by death, March 21, 1906. Death was preceded by a semi-comatose condition; a left otitis media had developed.

At the =autopsy=, it appeared that death was due to an early bronchopneumonia associated with acute splenitis and doubtless related to the otitis media of the left side. The body at large showed, aside from these acute lesions, a few chronic lesions, including slight scars of the left apex, and chronic adhesive pleuritis, chronic diffuse nephritis, and aortic and coronary syphilis. The aorta showed slight linear and nodular markings, with a single small dark ulcer in the upper thoracic region, but the aorta did not show the characteristic scarring which syphilitic aortas often show. The femoral marrow was of a dark red chocolate color. The thyroid appeared to be smaller than normal. A slight sacral decubitus had developed.

The description of the head (E.E.S.) is given in full on account of the encephalitic lesions shown. These encephalitic lesions may be summed up as follows:

Local cerebral =atrophy= and =sclerosis= of the frontal, orbital, and central regions, especially of the left operculum and left supramarginal gyrus.

Extension of sclerosis to hippocampal gyri with effacement of substantia reticularis alba.

Slight chronic internal =hydrocephalus=.

Granular =ependymitis= (especially of floor of 4th ventricle).

Compensatory edema of frontal and central pia mater.

Cerebellar sclerosis (culmen monticuli, lobus culminis, lobus cacuminis).

Spinal sclerosis (grossly evident in the posterior columns of the upper thoracic region and of the lumbar enlargement).

The details are as follows:

Head:—Bald on top. Hair =gray=. Scalp normal. Calvarium thin, deeply excavated by arachnoidal villi to right of vertex. Diploë absent. Dura closely adherent in bregmatic region. Dura of usual thickness. Sinuses contain cruor clot. Arachnoidal villi slight. Pia mater hazy and over sulcal veins porcelain white over all of vertex except occipital poles and over flanks (notably left). Thickened also around circle of Willis, over culmen monticuli and in posterior cerebellar notch. Edema of pia corresponding to atrophy of frontal and central regions. Cerebral atrophy most marked in orbital surfaces of both frontal lobes, in left area of Broca, and in left supramarginal region. The ascending branch and the ascending ramus of the posterior limb of the left Sylvian fossæ both readily admit the thumb by reason of atrophy of adjacent substance. Induration corresponds closely with atrophy, but is not more marked about the left Sylvian fossa. There is sclerosis of both hippocampal gyri, with loss of the substantia reticularis alba. The culmen monticuli and lobus culminis are firmer than the clival regions, and the lobus cacuminis is again slightly firmer than the clival region. Cerebellum a little softer than usual. Pia strips with usual readiness from all regions. The subpial region of the frontal lobes is a trifle grayer than that of the rest of cerebrum. Ventricles slightly dilated. Surfaces evenly sanded. Floor of fourth ventricle shows numerous coarse, closely set granules. Brain wt. 1200 grms. Cord shows a slight increase of consistence over one or two upper thoracic segments and in lumbar enlargement corresponding with a slight graying out of posterior columns. In places there is a suggestion of graying out also in lateral columns. A few calcified plaques in posterior lumbar pia.

Analysis of these details shows a number of lesions that characterize paretic neurosyphilis (among others, granular ependymitis, frontal atrophy, chronic leptomeningitis), but the lesions are more than merely frontal, extending as they do back as far as the postcentral regions on both sides, and even as far as the left supramarginal gyrus. The cerebellar involvement although frequent, can hardly be said to be characteristic in paretic neurosyphilis. The spinal involvement is characteristic of a case which is probably to be regarded as one of taboparesis; that is, of paretic neurosyphilis following a number of years after the establishment of tabetic neurosyphilis. The aorta is almost constantly affected by sclerosis in paretic neurosyphilis. The absence of diploë in the skull is not infrequent and the adherent dura mater is often found.

Microscopically, the tissues showed the characteristic lesions of PARETIC NEUROSYPHILIS; nerve cell destruction, fibrillar and cellular gliosis, lymphocytic and plasma cell deposits about the small vessels.

1. What are the clinical evidences of syphilis outside the nervous system? The brownish branny eruptions of the skin, the depressed scars and the scaly eruption on right heel and sole are very suggestive of syphilis. Such clinical evidences of syphilis are very important in systematic examination. Although the laboratory tests are of the utmost assistance in the diagnosis of syphilis, the clinical signs should not be neglected, and no physician should rest satisfied with laboratory signs alone. X-ray diagnosis of bone conditions sometimes succeeds when all other methods have failed.

=GUMMA of cerebral cortex verified by operation; death.=

=Case 27.= The presenting picture in the case of David Tannenbaum was that of deep dementia, in which condition the patient was brought to the hospital. There was a meagre history to the effect that about four months before admission, he had lost his job in a hotel through lack of further work. We heard that at this time he had begun to suffer with excruciating pains in the head; at first, worse at night, later, worse by day. It appeared that this pain, though it came and went, was chiefly localized on the left side of the head. For a fortnight, Tannenbaum had been dragging his legs, until finally he had become unable to walk at all.

_Pari passu_ with these developments, Tannenbaum had become mentally confused and irritable, and his memory had become untrustworthy. For several days before admission, an appearance of marked dementia was presented, with slow incoherent, or at all events, irrelevant words, and a complete disorientation for person. However, his vision had become so poor that it would have been hard for him to have recognized any one.

It appeared that the family history was entirely negative; that the patient was without education but had been physically very strong, and had been fairly successful at first in the junk business, and later in the clothing business; but latterly he had been less fortunate in the clothing business, and finally had to resort to work as a laborer around a hotel.

His wife had had eleven pregnancies with but one miscarriage. Nevertheless, out of the eleven pregnancies, there were now but four living children.

=Physically=, Tannenbaum was a rather small man; he was flabby and looked as if he had recently lost weight. The skin showed areas of pigmentation on the face and sides of the neck, and some dark copper-colored circular areas, marble-size, in the neck (syphilitic?). There was a slight radial arteriosclerosis. The heart was slightly enlarged with distant and indistinct sounds. There was a small pedunculated growth on the right side of the abdomen.

The pupils failed to react to flash-light but they reacted to sunlight. They both were slightly irregular but were equal in size, and reacted in accommodation. There was apparently almost complete blindness and extreme deafness. Arm-jerks and knee-jerks were absent; there was an occasional slight response of the left ankle-jerk, but the right ankle-jerk was absent; the left abdominal reflex was very feeble; the right absent; the cremasteric reflexes were absent, but there were no other abnormalities in the systematic examination. Hand grips weak; gait awkward, with right leg held somewhat flaccidly.

It was significant that percussion over the left frontal and parietal regions was able to elicit great pain. Either through the patient’s deafness or through sensory aphasia, spoken language was not understood. The serum W. R. was positive, the fluid W. R. negative.

=Diagnosis=: The clinical symptoms seem clearly to indicate syphilis. The local skull tenderness and impairment of vision might well suggest intracranial pressure. Uniting these suggestions, we might automatically arrive at a diagnosis of cerebral gumma. We have learned to be rather cautious of making a diagnosis of gumma of the brain through its mere rarity.

Decompression was suggested and executed. A deep growth resembling a GUMMA, in the view of the surgeon, was discovered. No attempt could be made to remove it. The patient died without recovering consciousness.

1. What is the significance of the negative fluid W. R. in this case of cerebral gumma? The W. R. producing substances not infrequently fail to appear in the spinal fluid from a gumma of the brain. The serum W. R. was positive in this case, but even the serum W. R. may be negative in cases of gumma, both of the brain and of the body at large. It must be remembered that the serum W. R. may be negative in paretic neurosyphilis (general paresis); the serum W. R. is even more apt to be negative in cases of gumma.

2. Is operative procedure to be advised in cerebral gumma? There are cases in which the acute and threatening symptoms of heightened intracranial pressure require operative treatment simply because the therapeutist cannot wait for the effect of antisyphilitic treatment. Moreover, antisyphilitic treatment of cerebral gumma is not always as successful as that of most syphilitic lesions.

3. Could the intracranial pressure be caused by other syphilitic lesions than gumma? A heavy meningitis may cause symptoms such as produced by an intracranial tumor. In such a case one will usually find evidences of inflammation in the spinal fluid. Cysts caused by syphilitic lesions may also produce identical symptoms.

4. What is the significance of cranial tenderness? Where sensitiveness to cranial percussion is not due to a scalp lesion it is very suggestive of a tumor underlying this point. A gummatous lesion of the cranium itself, may occur without causing pain or increased sensitiveness.

=CRANIAL NEUROSYPHILIS (focal syphilitic extraocular palsy) without mental symptoms.=

=Case 28.= A chef, Paolo Marini, 28 years of age, reported that on awaking one morning, everything appeared double to him and that his right eyelid had begun to drop. In the following month Marini had begun to feel weak and to have difficulty in swallowing, as well as at times difficulty in breathing. The diplopia was found to develop when Marini looked to the right. Mentally, the patient was in all respects normal, and no other physical signs were found except the diplopia and ptosis above mentioned. The W. serum test was positive, but the tests of the spinal fluid were negative.

=Diagnosis=: “CEREBRAL SYPHILIS.”

1. What is the anatomical cause of this condition? It is thought to be due in a number of cases to a small diffuse gummatous lesion at the _basis cerebri_. In the case of Marini this lesion appears to have been a little more extensive and to have interfered with the tenth and twelfth nerves also.

2. Why is the spinal fluid negative in such a case as that of Marini? Head and Fearnsides believe that intracerebral lues is characterized by a negative spinal fluid, under which circumstance one has always to consider the possibility of brain tumor or migraine in addition to the suspicion of syphilis.

3. What other causes besides syphilis should one consider for the sudden diplopia? Brain tumor, multiple sclerosis, cerebral arteriosclerosis, tuberculous meningitis, trauma and migrainous ophthalmoplegia, are not infrequently at the bottom of this condition. Cases also occur in which the etiology remains obscure, even at autopsy.

Under antisyphilitic treatment, Marini slowly improved.

=The SIX TESTS in TABETIC NEUROSYPHILIS (“tabes dorsalis”) may run milder than in paretic neurosyphilis (“general paresis”) and characteristically run somewhat like those of diffuse (meningovascular) neurosyphilis; in particular, the fluid Wassermann Reaction and the gold sol reaction are apt to run milder. The clinical course of tabes dorsalis is protracted and the prognosis as to life is good.=

=Case 29.= Mario Sanzi, 55 years of age, had been having what he called rheumatism since his 43d year. This rheumatism affected only the hips and legs, had at times been very severe, and for two years past had been almost constant. Before that time, pains had come at intervals, lasted a variable period, and suddenly disappeared. They were of knife-thrust character, and could probably be called “lancinating.” In a given attack, these pains would come at intervals of seconds or more. There was also a certain unsteadiness in locomotion and inability to control the vesical sphincter.

=Physically=, the patient was entirely normal so far as could be made out except =neurologically=. Argyll-Robertson pupils, absence of knee-jerks, and ankle-jerks, Romberg sign, and characteristic gait, left no cause for doubting the diagnosis of TABES DORSALIS. The blood and spinal fluid both proved positive to the W. R., though the W. R. in the fluid gave a negative reaction with 0.1 cm. and became positive with 0.3 cm. or more. The globulin was somewhat increased though less markedly so than in paresis. The gold sol reaction was “syphilitic” but weak. It is to be noted that the disease had run a 12–years’ course before a doctor had been consulted. The primary infection occurred at 32 years, namely, 11 years before the symptoms began. At the time of his primary infection, Sanzi had received several years of treatment, chiefly in the form of mercury by mouth.

1. What is the value of mercurial treatment of syphilis in the prevention of tabetic or other forms of neurosyphilis? “Fournier strove for many years to convince the medical profession that a syphilitic patient should be treated for at least two years after his infection, whether the syphilis seemed latent or patent. The method of treating only the symptoms he characterized as the opportunist method; treatment in the absence of definite symptoms the preventive method, as preventing the later manifestations. That prolonged treatment does prevent is shown by Fournier’s figures analyzing 2396 cases presenting tertiary signs. These he divides into three groups: Group I, comprising 1878 cases, or 78 per cent of the whole number, having no treatment or inadequate treatment—that is mercury for less than one year; Group 2, comprising 455 cases, or 19 per cent, having moderate treatment—that is, mercury for one to three years; and Group 3, comprising the remaining 19 cases which represent only 3 per cent of the whole number, having treatment for more than three years.”[7]

In the light of what we now know concerning latent neurosyphilis, it would seem well for patients to be followed from time to time with the W. R. on blood and spinal fluid after the supposed completion of the treatment of primary and secondary syphilis. The examination of the spinal fluid is not superfluous, as our experience with the so-called _paresis sine paresi_ abundantly shows. At the present day it is not good practice to assure a patient that he is cured after two years of ordinary mercurial treatment without resort to frequent spinal fluid tests, even though the serum W. R. be negative.

=TABETIC NEUROSYPHILIS (“tabes dorsalis”) is often quite ATYPICAL clinically and may even show no single symptom warranting the old clinical name “locomotor ataxia.”=

=Case 30.= Stephen Green is a case of TABES DORSALIS with active knee-jerks and without locomotor or muscle-sense disorder. When observed at the age of 45, it appeared that there were but two complaints: lack of control of the vesical sphincter and shooting pains in the legs. It appeared that the urinary disorder dated back ten years, when there had been difficulty in passing the urine. Sounds had been passed at the time; occasionally there had been incontinence during after years, ascribed by Mr. Green to the passing of the sound. However, the physician at that time stated that the incontinence was a symptom of tabes dorsalis. The incontinence had recently become worse, especially marked at night, though also occurring in the day; much worse during excitement, and very much worse after taking alcoholic drinks. Besides incontinence, there is also difficulty at times in passing the urine, as well as dysuria.