Читать книгу Neurosyphilis - Elmer Ernest Southard - Страница 8

1. Pons, normal except for focal infiltration of left fifth nerve.

2. Higher power view of infiltrated left fifth nerve.

3. Detail of infiltrated left fifth nerve, showing: 1, diffuse infiltration with mononuclear cells; 2, perivascular infiltration; 3, strands of relatively unaffected nerve fibers.

Microscopic appearances in Case 6. Extraocular palsy (focal meningeal syphilis, especially of left fifth nerve). Illustrates exquisite focality of the syphilitic process sometimes found, as well as its unilaterality (giving rise to asymmetrical symptoms and signs). Process in itself probably curable.

GUMMATOUS NEUROSYPHILIS (“gumma of brain”). Autopsy.

Case 7. Mrs. Lecompte was a woman of middle age, who, according to the history given by her son, had been entirely well until her final illness, which began approximately two years before admission to Danvers Hospital. The beginning of her trouble seemed to be chiefly headaches, which would last continuously for several days, or more than a week at a time. These headaches lasted throughout the course of the disease. In the morning, on arising, she would feel very dizzy, but this would pass away during the day. She had had a number of spells of unconsciousness, lasting about fifteen minutes. In these attacks she would breathe heavily, there was frothing at the mouth, twitching of the hands, and the eyes would roll about. Her memory failed gradually, her disposition changed and she became very irritable. Vomiting occurred almost every day, and at times was of a projectile character. She became hallucinated; the hallucinations were chiefly of a visual nature.

About four months before admission to the hospital, after one of her seizures, the entire right side was found to be completely paralyzed, and she complained that it was numb. At this time, she had difficulty with her speech. In a few days, however, she was able to talk correctly again, and in a week she was back at work, although the right side was weak and awkward. She continued to grow worse, and then began to have spells lasting several days, so that it became necessary to have her placed in a hospital.

On admission to the hospital, aside from obesity, the general viscera showed no points of special interest, and there was no evidence of any new growth outside of the nervous system. She was unsteady on her feet, standing with them wide apart. The gait was quite ataxic; the whole right side was weaker than the left and used more awkwardly. There was a paralysis of the right side of the face; the right angle of the mouth drooped; the right eyelid could not be closed but remained continuously open; nor could the right side of the forehead be wrinkled. Vision and hearing were not affected. She miscalled tastes and smells; whether this was due to aphasic difficulties or to cranial nerve involvement could not be divined. There seemed to be some difficulty in deglutition. The knee-jerks were markedly exaggerated; slight clonus was obtained but was not always present. Both pupils reacted well to light and distance and consensually. Sensation could not be readily tested. There was marked ataxia, especially with the eyes closed. The speech was thick and mumbling. The patient was unable to write or copy. Mentally the patient was quite dull; at times, stuporous; when aroused, was found to be entirely disoriented. Memory almost entirely absent. In general she showed herself to be very much confused.

She remained practically in this condition, even gaining in weight, for the following two years, when suddenly one morning, she had an epileptic seizure, vomited, coughed a great deal, with bleeding from the mouth and ears, and died in a few hours.

The symptoms in this case pointed to brain tumor. The only inconsistent thing was the long-continued life,—four years,—after the symptoms were observed. As she lived before the W. R. and spinal fluid tests were known, no light was gained in these ways. The post mortem examination showed the patient had a Gumma of the Brain.

The summary of the anatomical diagnoses at autopsy was:

Decubitus.

Lymphadenitis of the mesenteric nodes.

Chronic fibrous peritonitis.

Chronic fibrous myocarditis.

Pulmonary hypostasis.

Thrombosis of vein in right adrenal, with hemorrhage.

Syphilitic leptomeningitis.

Gumma of left hemisphere.

Focal softenings in the pons.

The anatomical description of the head (Dr. A. M. Barrett) is as follows:

The sutures in the calvarium are well outlined; diploë large in amount. The dura is diffusely but lightly adherent to the calvarium; it is very dense, especially over the left hemisphere. The meningeal arteries are thickened but not atheromatous. The sinuses contain a small amount of fluid blood and post mortem clot. The inner surface shows nothing abnormal. There is a great flattening of the convolutions of the left hemisphere, which is not the case on the right side. Over the convexity, the pia is thin and not abnormal except for some slight adhesions between the frontal lobes and the two lips of the Sylvian fissures. The pia at the base over the cisterna, pons, and medulla is thick, cloudy, and of a grayish gelatinous appearance. It is so thick that it is easily removable in a large piece.

The surface of the left hemisphere is dry, and the whole brain is flabby and bulges as if from internal pressure. A section through the hemispheres at the region of the optic chiasm shows a hard, firm area in the left hemisphere deep down in the white substance. It is about 2½ cm. in diameter, with a wavy border. The central part is of a silver-gray gelatinous-like appearance, with red spots and whitish streaks radiating from the centre. In the pons on the right side, in a plane passing through the posterior corpora quadrigemina, are two pinhead size softenings among the pyramidal fibres. The ependyma of the fourth ventricle is granular.

Microscopic examination of the tumor: The area evidently contains several central necrotic foci surrounded by zones of infiltration and proliferation, with bordering areas of nervous tissue showing secondary reactions. The necrotic area stains poorly. From the edge there are projections of reddish homogeneous bands, some intermixed with well-differentiated fibrillæ, probably glia fibrils. The bordering zone is densely infiltrated with lymphoid, plasma, and a few epithelial cells. The nerve tissue outside of this zone is spongy and infiltrated with lymphoid and plasma cells. There are a few scattered, shrunken nerve cells. In this zone and in the zone of infiltration near the necrotic area, there are scattered cells resembling giant cells. There are many obliterated vessels in the area, and other vessels show many infiltrating lymphoid and plasma cells in the walls. The examination of the specimen stained by the methods for bacilli of tuberculosis are negative. The growth is a classical gumma.

GUMMATOUS NEUROSYPHILIS (gumma of spinal meninges, “meningitis hypertrophica cervicalis of Charcot?”). Autopsy.

Case 8. John Wyman was first seen in his thirty-sixth year by Dr. James J. Putnam. He denied syphilitic infection and stated that the first symptoms had come four months before. He had begun to notice a numbness of the fingers, at first of the right hand and shortly thereafter of the left hand. After a few weeks there had been difficulty in walking, and a few weeks later headaches, especially on the right side, developed. Two weeks before he was first seen medically, he had begun to have a feeling of tightness or constriction in his arms.

It appears that micturition had been impaired early, that is to say, a few weeks after the initial sensory disorder had begun. A catheter was used for a time and improvement followed. Shortly before consultation retention of urine developed again, this time associated with rectal incontinence. The feet began to feel heavy and dead. Then the legs began to be increasingly weak so that the patient was almost bedridden. Vision appeared to be normal except that reading was followed by fatigue. The speech was also slow but the slowness could be attributed to fatigue.

Notes of Dr. Putnam’s physical examination are as follows: The patient lay in bed on the left side, without motion, and almost incapable of motion. The tongue was protruded, and there was no paralysis of facial muscles, or of the eye muscles (the right pupil had been reported to be slightly larger than the left). There seemed to be a disinclination to move the head, but with some effort it could be moved, and without pain. The arms and hands were held rigidly in median positions; many movements were possible, but all were imperfect and of slight amplitude. The fingers were flexed to a moderate degree, and could not easily be straightened, and there was, in fact, a general rigidity of most of the muscles of the body below the neck, and even, in some degree, of the neck. The immobility was so great that the general impression made was almost that of a patient with fracture of the spine in the cervical region. Even the breath, and especially the inspiration, was imperfect. The legs were more freely movable than the arms, but still the motions were very stiff and awkward, and of slight amplitude; with effort the whole leg could be lifted from the bed, and flexed or extended with moderate force. The right leg was rather stronger than the left, but the left hand and arm were stronger than the right. The sensibility was almost absent over the hands and lower part of the arms, and was impaired over the entire head and neck, except the forehead, the middle part of the face, and the nose. It is interesting to compare the conditions of the sensibility here present with those seen in cervical syringomyelia. The sensibility of the upper part of the forehead was less good than of the lower part, and there was slight impairment even over portions of the lower jaw. The sensibility of the left (stronger) arm was rather more impaired than that of the right arm, while on the contrary the sensibility of the left leg was better than that of the right leg, though the difference between them was not great. These statements apply to sensory tests by contact, heat, cold, and pricking. Knee-jerks were highly exaggerated, and likewise the wrist-jerks. All forced attempts at movements were attended by a high degree of muscular tremor, especially when the patient was fatigued or under emotional strain. The fingers especially were the seat of coarse tremor.

The remainder of this clinical description (courteously supplied us by Dr. Putnam) may be quoted. A second examination which included also a few facts not given in the first examination was made on the following March 28, 1905. This report says “the ends of the fingers became numb about June 1, 1904. Work was given up on July 3, and at that time the patient was walking very badly. No treatment was used and no satisfactory diagnosis made. In the course of July he improved somewhat, and during August he was able to ride out a little (these spontaneous improvements are of interest for the diagnosis). He went away from home for a short time, but from the time of his return, about the last of September, he grew worse rapidly, and fell into the condition above described, in which he was wholly unable to help himself, even to turning in bed. At times he had a great deal of pain in the neck and forehead. Antisyphilitic treatment was recommended, and for a time potassium iodid and other iodid preparations were given, but at first in relatively small doses (grs. 75 daily). Under this treatment the excretion of urine rose to four quarts daily as a maximum though sometimes the quantity was not so great.”

Under this treatment the patient began soon to improve, and continued doing somewhat better till about five months later. He became able to walk downstairs and out of doors, and regained considerable use of his hands. The quantity of urine passed became greatly increased by the use of the iodid.

About the middle of March he became worse again. A careful examination of the sensibility showed that in general the condition was much the same as that previously reported. The iodid treatment, with perhaps some mercurial, was resumed; the potassium iodid was given in doses which were increased up to 850 grains daily, although this maximum dose was taken only for about one week. This large quantity gradually impaired the sense of taste for the time being, and blurred his vision, but otherwise did him no harm. Under this he improved, so that he became able to run more or less, and went about freely, and attended to his business, though still retaining some stiffness in his movements.

This improvement continued until about two years later, when he again had a relapse, and was seen medically once more. His condition at this time was still a pretty good one, but the movements were stiff and awkward. The bin-iodid of mercury was advised, which was taken in doses of 9

25 grain daily. It will be remembered that this was long before the days of salvarsan treatment.

This was toward the end of June, 1907. Contrary to expectation, there was no material gain from this treatment, and the patient died early in October, without being seen again.

The autopsy was limited to the nervous system and the findings were as follows (Dr. A. R. Robertson):

Head: Hair abundant, fair, of fine texture and rather curly. Scalp of medium thickness and strips readily from calvarium. The latter appears normal and upon removal is of about the normal thickness. It lifts readily from the dura mater, except for the numerous attachments of Pacchionian granulations.

Meninges: The dura is smooth, moderately injected and shows no areas of thickening; it lifts readily from the pia-arachnoid. The pia-arachnoid shows discrete and in many places diffuse areas of opacity. There is a moderate amount of subpial clear fluid and the vessels are moderately injected. Over the anterior surface of the medulla and lower portion of the pons and largely confined to the right side there is a very marked thickening of the pia-arachnoid to which the dura is densely adherent. This thickening extends down anteriorly and laterally on the right side over the upper part of the cervical cord. The thickened meninges over the upper part of the medulla completely surround the right vertebral artery, shortly before it joins its fellow of the opposite side to form the basilar. Dissection of the arteries shows them to be patent and thin walled. Over the cerebrum and cerebellum the pia-arachnoid strips readily leaving a smooth surface. Section of the cerebral cortex, basal nuclei, pons and cerebellum show no gross lesions. The ventricles are moderately distended with fluid. The ependyma contains numerous small cysts. Section of the pons shows no lesions of the nervous tissue, but very marked thickening of the surrounding meninges as noted above.

Cord: Throughout the cervical and dorsal region the dura is quite tensely distended with an abundance of clear, light, straw-colored fluid. Upon snipping the dura this fluid escapes with a small spurt, as if under considerable pressure. The cord within, for the most part, lies free, but over the upper three or four centimeters of the cervical portion it is densely adherent to the dura anteriorly and laterally on the right side. Cross sections were made through the upper three or four centimeters of the cord, and over this area the cord is constricted by very marked thickening of all the meninges. The meninges here average from one to three millimeters in thickness. On the right side and somewhat anteriorly opposite the junction of the atlas and axis there is a single nodular, firm mass which on section shows a yellowish, firm center surrounded by very dense, pearl-gray tissue. The demarcation between the homogeneous yellowish centre and its surrounding gray tissue is very sharp. This nodule measures about 0.75 to 1 cm. in diameter. The adjacent cord is deeply indented by it. Below this nodule there is a translucent, grayish appearance of both posterior sensory columns which extends downwards and diminishes in intensity until it finally disappears in the upper dorsal region. This same appearance is well marked on the right outer margin of the upper cervical cord corresponding to the crossed pyramidal tract, and extends downwards diminishing in intensity until it disappears about the mid-dorsal region. The left pyramidal tract appears to be similarly but very slightly involved; section of the lower dorsal cord entirely negative. Microscopically, characteristic Gumma.

It is a question whether this case is one of the group described in 1871 by Charcot under the name of pachymeningitis cervicalis hypertrophica. Charcot did not regard his new disease as syphilitic, and it is very probable that syphilis is not responsible for all cases. Charcot, however, noted that his new disease was not incurable: he noted that the resulting paraplegia, although it might be very marked and accompanied by flexion of the leg on the thigh and although the paraplegia might have lasted a very long time, might end in recovery. Charcot thought that surgical intervention was necessary. He described three periods in the disease, the first or neuralgic (pseudo-neuralgic) was characterized by sharp pains in the neck and by the sensation of constriction in the upper part of the thorax. The second phase of the disease was, according to Charcot, the paralytic phase, in which a cervical paraplegia accompanied by muscular atrophy developed. Sometimes cases were found to remain in this paralytic phase and even to end spontaneously in cure. If the muscular atrophy was degenerative, then the atrophy was never replaced; but, according to Charcot, some cases of atrophy were simple and accordingly curable. If, however, the spinal cord itself became involved in the meningeal inflammation, then phenomena of transverse myelitis set in with a spastic paraplegia and involvement of the bladder and rectum. Muscular atrophy never developed in the legs, at least in typical cases.

Among the causes of this condition the following have been mentioned: cold, overexertion, alcoholism, tuberculosis and syphilis. Syphilis undoubtedly plays the major part. Even before the days of the W. R., observers, among whom may be mentioned Dejerine-Tinel and Pförringer, discovered syphilis in nearly all sufferers from pachymeningitis cervicalis hypertrophica.

It should be differentiated from caries of the spine and cord and meningeal tumors. The spinal fluid examination makes this somewhat easy.

Antisyphilitic remedies are indicated, and should be tried even when the etiology is obscure, if only as a therapeutic test.