III. PUZZLES AND ERRORS IN THE DIAGNOSIS OF NEUROSYPHILIS (continued 3)

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

275

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 276mother 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.

277

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.

278The 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.

279

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 280for 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 281nerve 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 282the 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.

1. Can we separate the symptoms of Addison’s disease from those of paresis in this case? The extreme cardiac weakness with a characteristic, small low-pressure pulse is in point. The asthenia and apathy are consistent enough with Addison’s disease as well as with paresis itself. It would also be possible to ascribe the gastro-intestinal symptoms to either disease.

2. Of what significance is the persistent thymus? Persistent thymus has been observed in a few cases of Addison’s disease, but that it plays any part in the symptomatology thereof is a matter of doubt.

3. How can the obesity be explained? It is of course of note that the thyroid gland was small, but microscopically there were no peculiar features in this gland.

4. Was the adrenal tuberculosis actually primary? Minute search failed to reveal evidences of tuberculosis elsewhere unless we regard the few adhesions binding the lower half of the lung to the chest wall as indicative of an old tuberculosis. In particular, the mesenteric lymph nodes were normal.

283

Neurosyphilis? Secondary stage of syphilis.

Case 81. Florence Fitzgerald, a woman 25 years of age, applied at the police station to be taken care of. She said she had been a prostitute for the last few months, was now ill, and wanted to reform. She appeared physically ill and was sent to the Psychopathic Hospital, where she remained at first almost mute, making answers chiefly by nodding the head. She gave the impression of daze or stupor, and in fact her condition was at first regarded as catatonic. This reaction, after a few days, changed and Florence became quite normal, giving a full account of her condition.

It seems that four months before going to the police station, she developed a chancre, which was locally treated. A careful physical examination showed a fine red macular eruption which was without much question a syphilitic roseola. The spinal fluid yielded a positive W. R. although other tests of the fluid were negative. Curiously enough, no physical sign of involvement of the nervous system could be discovered. We were inclined to regard the mental symptoms as partly due to the syphilitic intoxication, and partly due to a psychic reaction of the nature of defense. As for the positive W. R. in the spinal fluid, in early secondaries various observers differ as to the frequency both of the W. R. and of other changes, percentages being given that range from 25 to 90%. See case Caperson (45). It is of note that clinically there were symptoms referable to a syphilitic involvement of the nervous system; namely, marked headache and malaise. The headaches of the secondary period are frequently the result of meningeal involvement.

284

TABOPARETIC NEUROSYPHILIS (“taboparesis”); death from TYPHOID MENINGITIS. Autopsy.

Case 82. Frederick Estabrook was a salesman, who, be it noted, had never had typhoid fever or any disease remotely resembling typhoid fever. He had acquired syphilis at 19; had married at 22; was the father of two healthy children (no miscarriages); had had a certain disturbance of bladder and rectum, but remained a successful salesman to the age of 28, when advancing tabes confined him to bed for a time. At 30, mental signs of Paretic Neurosyphilis developed, and death followed at 32, after an acute illness of a week.

The details of the history after the first symptoms at 28 are as follows:

At twenty-eight patient lost control of limbs and was confined to the house about two months, under medical care. Three months later he had regained partial control of his limbs but had lost all control of his sphincters. After another month he had returned to work, but did not work steadily and seemed to have lost ambition. In the summer of 1905, his mind became obviously altered. He grew indolent and extravagant and given to buying expensive and useless articles. Loss of interest in things followed, together with loss of memory for recent events, lack of insight into illness, delusions of persecution by wife, irascibility followed quickly by crying. Before admission to hospital, he was euphoric, drawling and tremulous in speech, sprawling in penmanship, alternately depressed and exalted in manner. Knee-jerks were absent, gait ataxic, pupils stiff to light.

The family history was negative with respect to insanity. All the family were reported as nervous. A brother died of peritonitis at twenty-eight, a sister of pneumonia under twenty. Another brother and sister are living. Father and mother died of heart trouble at about sixty-seven and sixty respectively.

The patient was at high school one year and was a fair 285student. Considerable tobacco was used, and some alcohol. Intoxication denied. There was no history of typhoid fever or other acute disease.

The patient on admission was sallow, poorly nourished, and flat-chested, with a slight lateral curvature. There was slight dulness over right apex in front and in right upper back. Voice sounds were increased over right apex in front and over whole right back. The right chest showed bronchial respiration throughout. The respiration in front of right chest was of an interrupted character. The liver seemed moderately enlarged. The urine showed a very faint trace of albumin. There were a few small nodes in right groin and a scar on dorsum of penis.

Neurological Examination. Slight swaying in Romberg position. Slight tremor of protruded tongue and extended fingers. Pupils irregular, left slightly larger than right. Left pupil reacted to light consensually, but not directly. Right pupil reacted very slightly to direct light, not consensually. Knee-jerks and Achilles jerks absent. Ankle clonus absent, abdominal and cremasteric reflexes brisk. Sharp and dull points were recognized in the legs with numerous mistakes. Vocal and facial tremor. Speech slow and drawling. Test phrases repeated well if care was taken. Consciousness clear. Orientation perfect. Calculating ability preserved. Many words omitted in writing. Penmanship clear but shaky.

Hallucinations absent. Memory of recent events poor. Associations of a logical or defining type. Patient denied various statements in commitment papers and had little or no insight into the mental side of his disease—slight euphoria.

After a month’s observation the patient was removed to a quiet ward and set to work a few days in the scullery. One night he began to yell as if assaulted and said later that he had an idea that he was going to die. Before three months had passed he had become untidy, disorderly, and imperfectly oriented.

The general degeneration continued rapidly. One week before death the temperature rose to 103 degrees F., and the patient succumbed to what seemed clinically like a bronchopneumonia. Unconsciousness two days before death.

286Note with respect to history of typhoid.—Inquiries of his physicians, wife, employer, and brother tend to show conclusively that the patient never had a disease even remotely resembling typhoid fever.

The autopsy findings were as follows:

Acute conditions:

Hypostatic pneumonia, with early serofibrinous pleuritis and without lymph node swelling; enlargement of mesenteric lymph nodes; acute cerebrospinal leptomeningitis; multiple small hemorrhages of spleen.

Other findings:

Scar of penis; sclerosis of aortic arch (Heller’s type?) and slight coronary arteriosclerosis; calvarium thin and dense; dura mater thickened and adherent to calvarium; calcified arachnoidal villi; chronic cerebral and cerebellar leptomeningitis; atrophy of frontal lobes; granular ependymitis; sclerosis of posterior columns of spinal cord; emaciation; unequal pupils; slight parietal fibrous endocarditis, slight mitral sclerosis; gastro-intestinal atrophy; chronic cystitis; chronic abscess of prostate.

The description of the head findings is as follows:

Skin exceedingly loose, and the whole skull cap thinned. The diploë are absent. Adhesion with dura easily separated. The dura somewhat thickened, but not distended. Along the longitudinal sinus extensive calcareous granulations adhere to it. The longitudinal sinus does not contain blood, and the inner surface is normal in color. The pia is extensively thickened and opaque and a general subpial exudate exists which is more marked over the vertex where it lifts the pia from the brain surface to the extent of three centimeters in Rolandic, superior frontal, intraparietal, and mesial precentral sulci on each side. The arteries at base are free from atheroma. The temporal lobes are much bound down by adhesions, as is the cerebellum. Post mortem softening is evident. The hemispheres show no asymmetry, but the frontal convolutions are markedly atrophic. The corpus callosum is united to the cortex by old adhesions and has to be dissected away from it. Lateral ventricles contain some slight amount of cloudy fluid, and the pia along the vessels 287is opaque. Some granulations in ependyma. Brain weight, 1305 grams. Pons and cerebellum, 195 grams.

Cord.—Dura much thickened, and the pia corresponds to its appearance in brain with a like exudate. Cross sections of cord show sclerosis of posterior columns.

Bacteriologically the typhoid bacillus was cultivated from the meninges and from the swollen mesenteric lymph nodes. The blood was negative; the intestines were negative so far as lesions were concerned.

The microscopic examination confirmed the clinical diagnosis of General Paresis and of Tabes, since there was not only an extensive chronic encephalitis, with the usual lymphocytic and plasma cell deposit and irregular gliosis, but also a well marked posterior column sclerosis, not unusual save in its extreme degree.

It might be surmised that some difficulty would arise in distinguishing the effects of paretic meningoencephalitis from those of the more recent typhoidal process. The well-known tendency of typhoidal processes to escape polynuclear exudation, at least until frank necrosis has set in, gave rise to the idea that the two mononuclear pictures—that of general paresis and that of typhoidal processes—might be confusing.

The picture presented by the meninges was scarcely what might be expected. Although numerous mononuclear phagocytic cells are everywhere found, yet the predominant picture is that of a polynuclear exudation.

The polynuclear leucocytes occur in greatest numbers in the tissue spaces, especially in the meshes of the lumbar arachnoid and in the spaces of the frontal and paracentral pia mater. In the lumbar region of the spinal arachnoid wide fields occur in which the cells are almost one hundred per cent polynuclear leucocytes. In places phagocytic cells occur, and in a few fields, even in the open tissue spaces, the number of phagocytic cells may arise to fifty per cent. Edema is a considerable feature in the meninges. Fibrin is found chiefly in the cerebral meninges and appears in numerous delicate strands in the tissue spaces.

288

Moloch, horrid king, besmeared with blood

Of human sacrifice, and parents’ tears;

Though, for the noise of drums and timbrels loud,

Their children’s cries unheard that passed through fire

To his grim idol.

Paradise Lost, Book I, lines 392–396

289