Part 17

=Note.= THYROID: Weight 125 grams. Both lobes and isthmus enlarged. One lobe more than the other; lobe on one side measuring 6 × 4 cm.

Anatomical Diagnoses

Enlargement of thyroid gland.

Exophthalmos with dilated pupils.

Fatty degeneration of thoracic muscles.

Slight aortic sclerosis.

Dilatation of right heart.

Hypertrophy of left ventricle.

Slight tricuspid endocarditis.

Bicuspid aortic valve.

Hypostatic pneumonia.

Acute and chronic splenitis.

Fatty liver (central necroses?).

Acute nephritis.

Chronic gastritis.

Small breasts.

Axillary hair absent.

Petechial eruption of chest.

Varicose veins.

Chronic external adhesive pachymeningitis of left side.

Moderate swelling of right hemisphere with venous injection.

Slight occipital gliosis of both sides.

Slight gliosis of orbital and hippocampal gyri of right side.

Sclerosis with atrophy of occipital and hippocampal white matter of right side.

Gliotic lesion (1.5 × 2 × 2 cm. of right lenticular nucleus involving anterior commissure).

1. Was the exophthalmic goitre in Carrie Pearson due to syphilis? Unfortunately we have no clear proof that Carrie Pearson was syphilitic. She was stated to have been syphilitic by the physician who treated her before her commitment to Danvers Hospital. There is, however, no proof of syphilis, inasmuch as the patient died in the pre-Wassermann period.

2. Is the thalamic lesion probably syphilitic? No lymphocytosis or plasmocytosis characterizes the lesion, which is the only lesion of the sort in the Danvers collection. It would not do to call a lesion syphilitic just because it is _sui generis_. In any event, the clinical analysis of the case faced the claim of syphilis as an actual factor in the patient’s life and as a possible factor in the goitre.

=It is well known that the ARGYLL-ROBERTSON PUPIL is characteristic of the so-called “PARA-SYPHILITIC DISEASES” (“general paresis” and “tabes”); does this sign occur in other neurosyphilitic conditions?=

=Case 54.= Julius Kantor was a shoemaker of 35 years, who came to the hospital for treatment because his family physician had found a positive W. R. in Kantor’s blood serum. He had had a cough for a number of years, and during the last year a little blood had been found in the sputum; whereupon Kantor had been placed under active anti-tuberculosis treatment. The enterprising family physician had found the positive W. R. in the first days of his treatment for tuberculosis. There was, in fact, a history of a chancre nine years before, which had not been followed by any secondary or tertiary symptoms, and which had been but scantily treated.

There were no mental symptoms.

Kantor was =physically= fairly well developed and nourished. There were a few piping râles in the left upper chest, both in front and back, and also a slight dulness with increased vocal and tactile fremitus. No tubercle bacilli, however, could be found on repeated sputum examination.

=Neurologically=, the pupils were myotic and both showed the Argyll-Robertson reaction. There were no abnormal reflexes whatever, and there was neither ataxia nor speech defect. Not only the blood but also the spinal fluid W. R. proved to be positive; there was a marked increase in the albumin and globulin; there was a gold sol reaction of the syphilitic type, and there were but three cells per cmm.

1. In view of the headache in case Kantor, what other causes of headache are to be considered? It is certain that irritations of the dura mater can produce headache, and the physiological observation of the sensitiveness of the membranes and the non-sensitiveness of the brain substance is an ancient and classical observation. Internal hemorrhagic pachymeningitis produces severe headache. The relations of this disease to trauma, to arteriosclerosis, and possibly to syphilis (alcohol perhaps should also be considered) in certain instances have not been entirely cleared up. Syphilitic headaches are, according to Lewandowski, dependent also upon a dural affection or upon a periosteal affection. The headaches of brain tumor are also commonly related to dural conditions, either directly due to the pressure of the tumor itself, or indirectly to the heightened intracranial pressure consequent upon the tumor. It is clear that the tension under which the dura mater lies is not always localized in the region of a brain tumor or a syphilitic lesion. Head has claimed that brain tumor produces headaches of two kinds, according to whether the disease affects the dura mater or is dependent upon an increase of pressure in the brain. It does not appear that the pia mater has any relation to headaches, but meningitis, in which the inflammation is confined to the pia mater, is nevertheless associated with headache; the headache is here supposed to be due to the increase in brain pressure, and thus actually to an effect wrought upon the dura mater. Vasomotor disorders and various types of cephalic hyperemia are thought to produce a kind of headache, but Lewandowski calls this kind of headache somewhat in question. Reflex headaches are stated to be produced indirectly by a process of radiation from interior lesions in the brain. There are certain headaches called nodal headaches (_Schwielen-Kopfschmerz_). Hypermetropia, caries of the teeth, adenoids, and diseases of the nose and axillary cavities, to say nothing of thoracic and abdominal diseases, are also counted among conditions that may produce headaches. In this connection, Head has claimed differential zones of headache corresponding to certain diseases.

The brain itself may produce headache through intoxications, through conditions produced by a variety of diseases; may follow neuroses. Alcohol may produce headaches in some persons even when it is taken in very small doses. Certain uremic cases yield headaches, as do also gouty and chlorotic conditions. According to Lewandowski, the headaches of arteriosclerotics are due possibly to vasomotor disturbances in the membranes, or one may think of nutritive cerebral disorders. A peculiar form of headache is that of fatigue after mental work, allied to which is the neurasthenic headache; constitutional headaches have been assumed to occur, to say nothing of hysterical headaches. There remains also the important question of migraine, for which a vasomotor explanation has been proposed.

2. Was Kantor suffering from tuberculosis of the lungs? The hypothesis of lung syphilis ought certainly to be very seriously considered. Upon repeated sputum examination, no tubercle bacilli have yet been found.

3. Is Kantor a case of general paresis? In the absence of mental symptoms, and in consideration of the mildness of the reactions, it is certainly not easy to make the diagnosis of general paresis. However, the diagnosis of tabes dorsalis is not justified either. Accordingly, we may answer our question: whether the Argyll-Robertson pupil occurs in other neurosyphilitic diseases, by pointing out that in the case of Julius Kantor, as in the case of Henri Lepère (105) and Frederick Stone (106), the Argyll-Robertson pupil has been found in syphilitic conditions that are neither typically paretic nor typically tabetic.

=Does the Argyll-Robertson pupil necessarily indicate neurosyphilis?=

=Case 55.= Daniel Falvey, 44 years of age, was an almshouse transfer to the Danvers State Hospital in the year 1904, when the principle of state care was adopted in Massachusetts. As in most of the almshouse transfers of that day, little could be discovered as to antecedents. He had been a mill-worker from the time of his immigration in 1890, at 30 years of age. He had been somewhat alcoholic. There was a shock some 17 months before his death, which occurred about seven weeks from the date of transfer.

Not only was he unable to walk unsupported, but when supported there was a slight dragging of the left leg and the gait was noted to be somewhat propulsive. The tongue and hands were tremulous, and the left grasp was somewhat weaker than the right. Both knee-jerks were increased although neither more than the other. There was no sensory disorder.

Although but 44 years of age, Falvey presented the appearance of a much older man. His heart was somewhat enlarged and there was a degree of peripheral arteriosclerosis. On the whole, no special attention was attracted to this case clinically and he was regarded as an example of arteriosclerotic dementia, like many another among the transfers. However, we owe to Dr. H. M. Swift the important observation of the Argyll-Robertson pupils. The case was studied long before the Wassermann method was available, and is here reported merely to call attention to the fact that the stiff pupils may have other neural origin than neurosyphilis.

The autopsy material in the case was worked up by one of the authors.[11] The autopsy had been performed by Dr. A. M. Barrett, who found on section through the brain stem at the anterior border of the pons a mass springing from and continuous with the pineal gland, lying in the third ventricle and the aqueduct of Sylvius. Upon further study, this mass was found to begin posteriorly in the pineal body itself, from which the mass could hardly be told in the gross except by an injected border.

This mass proved upon microscopic examination to be a psammoma, which histologically resembled a glioma rather than a sarcoma. Throughout the mass there was a variable content of fibrillary intercellular substance having the histological reactions of neuroglia fibrillæ. The histological details (mitosis, large giant cells with multiple nuclei, etc.) do not here concern us. We deal with a neoplasm springing from the pineal gland growing on the posterior half of the third ventricle, the anterior orifice of the aqueduct of Sylvius, and the space between the velum interpositum as far back as the posterior corpora quadrigemina. There is no evidence in the body of old syphilis; although it is possible that the stiff pupils were neurosyphilitic, it seems probable that they were related to the pineal tumor. At all events, there are in the literature evidences that the pineal-quadrigeminal group of tumors and other lesions may bring about pupillary disturbances. On this account, we here include the case. The tumor hardly led to an error in diagnosis since neither neurosyphilis nor brain tumor was at all expected clinically.

1. Can alcoholism produce identical results? See Case Murphy, (60), one of alcoholic pseudoparesis.

2. What is the nature of stiff pupils? A pupil is called stiff in the sense of the Argyll-Robertson pupil if it fails to react to illumination either of itself or of the other eye and at the same time if it reacts properly in convergence and accommodation. Of course the stiffness of a blind eye must not be regarded as an Argyll-Robertson pupil. In a case of right-sided Argyll-Robertson pupil, therefore, the left pupil reacts properly both to direct illumination of itself and to illumination of the right eye, but the right eye fails to react to illumination of either eye. Such an Argyll-Robertson right pupil will remain of the same width both in darkness and in light. Clinicians agree that the Argyll-Robertson is diagnosticated rather too frequently than too seldom, and this by reason of the fact that a sluggishness of light reaction is interpreted as stiffness. The sign, as is well known, has come to be regarded as almost pathognomonic of tabetic or paretic neurosyphilis. Nonne, however, has found among 510 cases of alcoholism, nine instances of Argyll-Robertson pupil and 19 cases of sluggish light reactions. The pathological anatomy of this sign is still doubtful although a number of schematic accounts are available; among hypotheses, one may think of an elective effect of the tabetic or paretic degeneration upon reflex collaterals. The explanation would then resemble that for absent knee-jerks and kindred reflex disorders. We should then hypothesize a loss of the finer processes of the terminal arborizations about the cells of the nucleus of sphincter nucleus iridis. However, the situation of the sphincter iridis has not yet been absolutely determined.

When a pupil is said to be entirely stiff it means that it reacts neither to light nor accommodation. This condition not infrequently follows the partial stiffness or Argyll-Robertson reaction.

3. Is the Argyll-Robertson pupil more tabetic than paretic? This has been claimed at times, but in point of fact, the Argyll-Robertson pupil is very frequent in paresis, and so also are posterior column changes. According to statistics of Bumke, 36% of tabetics fail to show the Argyll-Robertson pupil, and 38% of paretics. When, however, finer methods, such as those standardized by Weiler, with photographic records, are employed, the number of cases without at least a tendency to the Argyll-Robertson pupil becomes much smaller.

In connection with the important question as to the classical Argyll-Robertson pupil and pupillary sluggishness to light, it may be inquired what are the ocular signs in neurosyphilis? Joffroy has tabulated the signs in 300 general paretics as follows:

Sign. No. of Per cases. cent. Alterations of light reflex 235 78 Inequality 205 68 Abolition of light reflex (bilateral or unilateral) 156 52 Abolition of light reflex (bilateral) 133 44 Irregularity of pupil 117 39 Irregularity of both pupils 109 36 Diminution of light reflex 108 36 ditto (bilateral) 79 26 Alteration in accommodation reflex 79 26 Diminution of accommodation reflex 52 17 Mydriasis 41 13 Myosis 40 13 Diminution of light reflex (unilateral) 35 11 Abolition of accommodation reflex 35 11 Diminution of accommodation reflex (bilateral) 29 9 Abolition of accommodation reflex (bilateral) 26 8 Diminution of accommodation reflex (unilateral) 23 7 Fundus changes 21 7 Vascular changes 16 5 Abolition of accommodation reflex (unilateral) 12 4 Paresis of the third nerves 10 3 Ptosis 9 3 Irregularity of one pupil 8 3 Nystagmus 7 2 Visual acuity lost 7 2 Atrophy of disc 6 2 Total blindness 5 2 Paralysis of the fourth nerves 1 1

=Can neurosyphilis exist in the absence of positive findings in the spinal fluid?=

=Case 56.= There was no great difficulty in setting up a diagnosis of general paresis in the case of James Burns, a mechanic of 31 years of age, who came voluntarily to the Psychopathic Hospital for treatment. The point in Burns’ case was that the spinal fluid proved entirely negative in all respects despite the fact that the serum W. R. was positive, and despite the following facts of history and mental examination.

The patient claimed syphilitic infection seven years before, namely, at 24 years of age, and also claimed that he had infected his wife, who was in fact at the time undergoing antisyphilitic treatment. He complained of insomnia, worry, depression, hypersensitivity to noises (such as those made by his own children), thoughts of suicide, and amnesia. The amnesia, however, might be regarded as subjective since our tests failed to show amnesia. Nor was there any diminution in arithmetical ability. Despite the patient’s claim that he had been “way off in his way of thinking,” there appeared to be no delusions. Beyond a certain flightiness in conversation, we could hardly get any evidence of psychosis unless of the neurasthenic order.

=Physically=, however, the left pupil failed to react to light though it was found to react to distance, and the right pupil exhibited a diminution of its reaction to light. There was no ataxia of gait, yet there was a complete Romberg reaction. There was a moderate tremor of the hands and of the tongue. Otherwise there were no reflex disorders upon systematic examination, nor was there any demonstrable disorder in the rest of the physical examination.

1. What is the diagnosis in the case of James Burns? On the whole we agree with Nonne, that negative spinal fluid findings (of course, in the absence of treatment) preclude the diagnosis of general paresis. The symptoms might possibly be explained, however, by means of a localized syphilitic involvement of the cerebrum, no cells or products of inflammation having penetrated to the spinal fluid. According to Head and Fearnsides, this condition may be found especially in the anterior or middle fossa. Accordingly, going upon these views of Nonne and of Head and Fearnsides, we should be entitled to make, perhaps, a diagnosis of cerebral syphilis.

2. What is the significance of the Argyll-Robertson pupil in James Burns? Nonne states that if one follows cases with Argyll-Robertson pupil over a sufficient period of years, they one and all eventuate in active symptoms of cerebrospinal syphilis (not necessarily of the cortical type), and this despite the fact that the pupillary change may have been present a number of years before any other symptom had developed.

=Neurosyphilis (“DISSEMINATED ENCEPHALITIS”) within seven months of initial infection. Autopsy.=

=Case 57.= We borrow the main features of a remarkable case examined at the Danvers State Hospital clinically by Dr. H. W. Mitchell and reported elaborately by Dr. A. M. Barrett. This case, whom we shall call John Summers, acquired syphilis at about the end of the third week in May, 1902, and consulted a physician on June 12, at which time a characteristic initial lesion of syphilis was plain. Summers was excessively alcoholic at times and was not seen by a physician again until July 2, just after an alcoholic debauch. At this time there was ulceration of the primary lesion, and a papillary eruption had developed over the arms, chest, abdomen, and legs. Mercurial treatment and mixed treatment were given. Arthritis occurred but disappeared with increased dosage.

About six months after infection, the patient developed severe headaches, hardly controllable by treatment. Amnesia and a certain stupidity, with neglect of personal habits, and even of eating, developed, whereupon Summers was admitted to the Danvers Hospital, December 11, 1902. He weighed 124 pounds, was extremely feeble, with dull and expressionless face, coarse purposeless movements of arms; left pupil larger than right; right external strabismus and ocular ptosis; increased knee-jerks, crossed adductor reflex, coarse tremors of arms and hands; and extreme clouding of consciousness. It was doubtful whether the pupils were stiff to light or not.

The patient died on the ninth day, December 18, in a state of coma. After admission, his stupor had become more marked; there had been incontinence of urine and fæces, and the patient could be aroused only by loud tones. Difficulty in swallowing had developed; the right-sided ptosis had become more marked, and muscular twitchings had developed on the right side. When the left leg was pinched, there was twitching of the left leg and arm. There was slight spasticity of the right arm and leg. An examination upon the day of death definitely showed a lack of reaction of the pupils to light.

Case 57. Neurosyphilis (“disseminated syphilitic encephalitis” of A. M. Barrett), fatal seven months from initial infection. (Photographs by A. M. Barrett.)

Dr. Barrett was able to find in the literature a case of Bechterew which histologically resembled his own case, but though in the instance reported by Bechterew the first symptoms developed within the year following infection, death did not occur until two years later.

In view of a total duration of symptoms clearly not over seven months, it is interesting to inquire how far microscopic brain changes could have proceeded. Neither calvarium nor dura mater showed changes. There was a slight haziness of the pia mater over the convexity, but the pia mater over the base (especially below the cisterna and from thence spreading out over the pons and into the fissure of Sylvius) was not only hazy but definitely thickened and hyperæmic. The thickening was most marked about the root of the right third nerve (corresponding with the eye findings in life). There was also a macroscopic thickening of the left Sylvian artery. Section of the brain showed nothing abnormal except a small area among the pyramidal fibres of the right side of the pons, where there was a single hemorrhagic area about 7 mm. in diameter around which there were small punctiform hemorrhages. (Compare twitchings of left leg and arm upon stimulation of left leg, and note also the muscular twitchings and slight spasticity of right leg and arm noted just before death.) This case was examined and reported upon in 1905. We learn from Dr. Barrett that a re-study of the case with modern methods has failed to demonstrate a spirochetosis.

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