Part 22

=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, salvarsan 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.

=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.

The 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 cases 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.

3. How often is the central nervous system involved in hereditary syphilis? An interesting table bearing on this point is presented by Veeder.[16] The table concerns the lesions in various parts and systems of the body in 100 cases of late syphilis. It appears that in 47, or approximately one-half of Veeder’s series of 100 late cases, the infection developed some form of lesion of the nervous system. As Veeder remarks, this result runs counter to the common statements of pediatricians, notably of Holt.

Bones: Periostitis tibia 4 Periostitis skull 1 Osteomyelitis 1

Joints: Acute arthritis knee 8 Acute arthritis ankle 1

Skin: Macular eruption 1 Condyloma anus 3 Gummata 3 Alopecia 3

Eye: Interstitial keratitis 24 Choroiditis 1

Ulcerations: Nasal 2 Laryngeal 1 Pharyngeal 1

Central Nervous System: Mental deficiency 23 Cerebrospinal syphilis 14 Hemiplegia 6 Epilepsy 5 Spastic paraplegia 4 Chorea 2 Hydrocephalus 2

Miscellaneous Conditions: Ozena 1 Enlarged spleen (only symptom) 1 Torticollis 1 Aortitis 1 Obscure abdominal pain 1 Obscure pain in legs 2 Endarteritis obliterans 1 Paroxysmal hemoglobinuria 1 Raynaud’s disease 1 Hutchinson’s teeth 4

=Juvenile paretic neurosyphilis. Quadriplegia.=

=Case 78.= Gridley Ringer, 15 years of age, had the facies of a congenital syphilitic, including Hutchinsonian teeth, rhagades of the face, and the so-called Olympic brow. No secondary sexual characteristics had developed. There was a marked speech defect. =Mentally=, Ringer was a low-grade imbecile. He had been born at full term, and delivery had been normal. There had never been other pregnancies. He had never developed normally.

The father admitted syphilis 23 years before, namely, 8 years before the birth of his son, but the father had been treated for several years and had been declared cured.

1. What would be expected in the spinal fluid of this case? Without the history, it would perhaps be impossible to say whether the case was one of a quiescent imbecility or one of juvenile paresis. The spinal fluid of the juvenile paretic gives a picture identical with that in the adult. The spinal fluid in this case showed a positive W. R. (as did also the serum), a marked increase of albumin and globulin, 115 cells per cmm., and a “paretic” gold sol reaction. Accordingly, the diagnosis of GENERAL PARESIS was made.

2. What is the prognosis? The prognosis of juvenile paresis is currently regarded as entirely grave. There is probably less hope for improvement in juvenile paresis than in the acquired paresis of adult life, since it seems to be a general principle that congenital syphilis is always more difficult to cure than acquired syphilis.

This case had seizures a few months after initial observation, and the seizures were followed by a transient right hemiplegia. This right hemiplegia was shortly followed by a left hemiplegia, which remained permanently. Moreover, a few weeks later, a right hemiplegia again developed, leaving the patient with complete paralysis and aphasia. Death followed in six weeks.

3. What effects were shown in the parents? Following up the parents was rewarded by the discovery that the mother was suffering from nerve deafness, probably of syphilitic origin, and that the father had recently begun to suffer from what he considered rheumatism, but which on examination was shown to be tabetic neurosyphilis (“tabes dorsalis”). This family again supports the hypothesis that there is a strain of spirochetes especially prone to attack the nervous system. Here it would seem that the syphilis acquired by the father had infected the mother and been transmitted to the son. In all three infected by the same strain or strains of organisms the nervous system was involved. It is difficult, nevertheless, to explain on this hypothesis why in one case the disease took the form of tabes dorsalis, in the second, eighth nerve involvement and in the third, paresis. This question of strains is really more than academic because it enters deeply into the question of treatment, as well as that of the suggested increased viability of the neural strain.

=Is there a relation between epilepsy and juvenile neurosyphilis?=

=Case 79.= John Doran fell off the rear of an ice-wagon, at six years of age, and shortly afterward developed fits. It appears that John was not unconscious at the time of his fall, but that he complained of headache. Although the convulsions were fairly frequent at first, it appears that they later became rare and occurred only when the patient got into a temper. At the stage of exhaustion after violent excitement, John would fall.

=Physically=, at 9 years a fair development and nutrition were evident. There was a great exaggeration of the frontal bosses; the nose could not be said to be typically saddlebacked, yet there was a suggestion of a sinking of the bridge. The teeth slightly suggested the Hutchinsonian type, but only slightly. There was a slight roughening of the tibia, and there was a slight scar over either knee. The patient graded according to the Binet scale at 9 years, and he was regarded as definitely feebleminded.

The family physician states that, according to his information, the father contracted syphilis when the child was between three and four months of age, and that the mother also was infected at this time. However, the child had not been suckled except immediately after birth, and there had been no evidences, according to the family physician, that John had acquired syphilis.

Ordinarily, one might content himself regarding the case of John Doran as one of idiopathic epilepsy with mental defect or deterioration. However, the frontal bosses, suggestive teeth, the flattened bridge of the nose, the roughened tibiae, and the old scars, though singly not of great significance, collectively make one suspicious. Despite the family physician’s belief that John could not have acquired syphilis from the parents, the infection seems entirely possible despite the fact that no symptoms developed early thereafter.

The W. R. in this case proved positive in both blood serum and spinal fluid.

1. What is the relation of trauma to this case of JUVENILE NEUROSYPHILIS? Probably none.

2. What would be the effect of treatment? For a number of years John Doran was lost sight of. He was, however, treated, according to our information, with intraspinous injections of salvarsanized serum, whereupon his convulsions shortly ceased. He has been recently examined mentally once more, and still grades as feebleminded. He still has violent outbreaks of temper.

3. Is such a case as Doran typical? Shanahan has investigated conditions at Craig Colony. There were 22 out of 886 epileptics (at Craig Colony) or 2½%, who showed a positive W. R. Nine of these cases were regarded by Shanahan as cases of epilepsy actually caused by syphilis. Viet had found 7%, and Bratz and Lüth 5% of constitutional epileptics to be syphilitic, but the data of these German authors were obtained before the era of Wassermann tests.

=Adrenal tuberculosis complicating juvenile paretic neurosyphilis (“juvenile paresis”). Autopsy.=

=Case 80.= When James Arnold appeared at the Danvers Hospital in his 22d year, he looked as if he were but 12 or 14 years of age. He was excessively fat but of fair muscular development. The left eye diverged outward, and the left pupil was smaller than the right. An odd feature was a high degree of pigmentation of the skin of the genitalia and the groins (the axilla, the mammillary areas, and the oral mucosæ were free from pigmentation). =Physically= speaking, the patient was practically normal. =Neurologically=, however, there was much of interest, in the light of which the clinical history was of value.

It seems that after an apparently normal early childhood, the boy had begun, at the age of 11, to experience difficulty in carrying out every-day school tasks; and after this his mental capacity had slowly but progressively deteriorated. The deterioration was not merely intellectual, but the boy became dishonest and untrustworthy and developed a number of untidy and uncleanly habits, behaving at the age of 16, as the parents stated, like a child of six.

In his seventeenth year, the boy had been taken with a severe attack of what was regarded as an “attack of indigestion.” This attack ushered in a gradually developing muscular weakness, especially involving the limbs. By the age of 21 he had become irritable and the paresis was so extreme that the patient was unable to get in or out of a carriage.

This generalized muscular weakness was plain upon admission to the hospital though there seemed to be no actual paralysis. The patient was unable to walk in a straight line and Romberg’s position could not be maintained. Marked tremor was present in the hands and lips. There was bilateral impairment of vision and nystagmus. Reflexes and sensations normal. Speech was markedly affected, all syllables being very much slurred. School knowledge and memory for both recent and remote events very poor. The patient’s habits were very untidy. He was very emotional, easily made to laugh or cry; and in behavior, extremely childish.

Two months after his admission to the hospital, the weakness suddenly became extreme. He was constantly nauseated, refusing food. The face and hands were cyanosed and the heart’s action rapid, weak, and irregular. This attack lasted for a week and was followed by a period of improvement, during which, however, he still remained very weak and apathetic.

One month later he again became so feeble that he was unable to raise himself in bed. He complained persistently of feeling very “sick.” His temperature was elevated and there occurred the same train of circulatory symptoms observed previously, _viz._, rapid and tumultuous action of the heart, with cyanosis of face and extremities. He soon became unconscious, remaining so until his death, which occurred on the seventh day of the acute attack.

This case was under observation before the days of the W. R., yet clinically the case had been diagnosticated JUVENILE PARESIS. There was no history of the acquisition of syphilis or any likelihood of its acquisition. Considered clinically, many of the classical features described by Addison were present, _viz._, marked asthenia and apathy; severe and frequent gastro-intestinal symptoms (the disease probably commencing with the attack of so-called “acute indigestion” six years prior to patient’s death); attacks of extreme cardiac weakness with the characteristic small, low-pressure pulse. On the other hand, pigmentation of the skin (usually the most striking clinical feature) was limited to the external genitalia, these being colored a deep brown.

The most striking feature found at autopsy was a bilateral adrenal tuberculosis (caseation, giant cells, lymphocytosis, tubercle bacilli). The thymus gland was persistent (7×5×.5 cm.), whereas the thyroid gland was smaller than usual. The brain showed macroscopic and microscopic features entirely consistent with the diagnosis of general paresis, including lymphocytosis, plasmocytosis, irregular degrees of nerve cell destruction, and gliosis, with an especially characteristic microscopic picture in the frontal regions.

It may be of note to consider the degree of change undergone by a brain in 11 years or more of deterioration, and the following description of the head findings is therefore included:

=Head=: Hair abundant, dark. Scalp normal. Calvarium, weight 435 gm., transparent in bregmatic region only, elsewhere thick and dense. The average thickness of the vertical plate of the frontal bone is 7 mm. The frontal bone shows a moderate thickening and hardening of the inner table with obliteration of diploë. Dura mater moderately adherent to the bregmatic region of calvarium. Arachnoidal villi moderately developed. Sinuses not remarkable. Pia mater shows a moderate focal thickening with opacity, especially along sulci. Vessels well injected. =Brain=: Weight, 1200 gm. The brain shows marked focal variations in sulcation and consistence. Spread on a board, the right hemisphere is obviously somewhat bigger than the left. There is a difference of only 0.5 to 0.75 cm. on measurement of the greatest circumference of the cerebrum, taken from the median line superiorly to the median line inferiorly, but the right hemisphere is throughout slightly more convex than the left. Both postcentral gyri are much narrowed in their superior portions, and the sulci posterior thereto are deeper than the other sulci of the hemispheres. The sulci of the orbital surfaces are asymmetrical and, on the left side, show a tendency to microgyria. The cerebral hemispheres as a whole show a remarkable tendency to slight protrusion of the border gyri; especially those of the two poles, of the free edges along the great fissure, and most strikingly the gyri at the boundary line between the inferior and lateral surfaces. This _marginal prominence_ is slight but obvious and is emphasized by a slightly paler color in some regions. The cerebrum shows a general induration which is greatest in the frontal tips and along the inferior borders of the lateral surfaces of the hemispheres, especially right. The orbital surfaces are firm, especially anteriorly and externally (prefrontal); the tips of the temporal lobes are firm, and the superior temporal gyri are firmer than adjacent gyri. The postcentral gyri are indurated more than the other gyri of the superior surface. The hippocampal gyri are likewise firmer than adjacent gyri.

=Cerebellum and pons=: Weight, 145 gm. The inequality of the two hemispheres is more marked than in the case of the cerebrum.

Greatest lateral diameter; left, 4.5 cm., right, 5.5 cm.

Anteroposterior diameter adjacent to notch: Left, 5.8 cm., right, 5.5 cm.

There is no appreciable difference in depth. The diminution in volume appears to be chiefly at the expense of the right clivus. The inferior surface is firmer than the superior. The laminæ adjacent to the horizontal fissure are firmer than the remainder of the cerebellum. The pons is small.

There was also a lateral curvature of the spinal column, as well as characteristic adhesions between spinal dura and pia mater which are always suggestive of syphilis. For the rest, there were few findings of note: some adhesions of the left pleura, hypostatic congestion of the lungs, tracheitis, and chronic gastritis. There were four lobes of the right lung but it is doubtful whether this should be regarded as a stigma.