Читать книгу Neurosyphilis - Elmer Ernest Southard - Страница 7

Apparent new formation of small blood vessel. Photographed by Dr. A. M. Barrett.

Rod cells (Stäbchenzellen) in paretic neurosyphilis. Photographed by Dr. A. M. Barrett.

Granular ependymitis—microscopic appearance of a marked example of “sanding” of ventricle.

Characteristic and constant in paretic neurosyphilis is the Plasmocytosis and Lymphocytosis, Perivascular in distribution about small cortical vessels. There is also a characteristic (though characteristically less prominent) Plasmocytosis and Lymphocytosis, Meningeal in distribution. The pleocytosis of the spinal fluid, almost constant though variable in amount in life, is an indicator of the meningeal picture and less directly of the parenchymatous picture.

Granular Ependymitis (“sanding” of ventricle floors) is characteristic and may be regarded as part of the parenchymatous picture. This ependymitis is an indicator how chemical changes could be readily produced at least in the ventricular fluids, since the limiting membranes of the nerve tissue are here subject to multiple breaks. The “sanding” is a neuroglia reaction to these multiple small breaks (Weigert’s explanation).

Parenchymatous losses have led to Atrophy and Sclerosis, of very varying extent in different parts of the encephalon. The atrophy is characteristic in paretic neurosyphilis, but by no means constant. Numerous cases have come to autopsy without clearly defined gross atrophy. Sclerosis is also characteristic and even more frequent than atrophy, doubtless because sclerosis represents an earlier phase of a process eventuating in gross atrophy.

A Tabetiform Picture characterizes the spinal cord, but in this case the tabetic clinical picture did not precede the paretic clinical picture. We are consequently to regard the tabetic spinal process as incidental and on all fours with the Cerebellar and Pontine Atrophy.

VASCULAR NEUROSYPHILIS (“syphilitic cerebral thrombosis”). Autopsy.

Case 4. James Pierce was an almshouse transfer to the Danvers Hospital in his fiftieth year. He died three years later. The accompanying brain pictures demonstrate so extensive a lesion of the left hemisphere that it is of great interest to determine if possible the genesis and course of his disease. It appears that syphilis had been acquired somewhere about the age of 38 or 40, so that the total duration of the process was between 13 and 15 years. In Pierce’s forty-third or forty-fourth year, he had a shock while walking in the streets of his native city, whereupon he was subsequently transferred to the Danvers Hospital, whose data have been summed up as follows (we are obliged to Dr. Charles T. Ryder for these data):

Neurological examination: Neuromuscular condition: Barely able to walk or stand without assistance; hemiplegia of right side; swings foot out and drags toe out and around in attempting to walk. Right hand held by side, flexed at right angle; fingers contracted and thumb thrown across palm. Can lift arm from side; practically no movements of forearms or fingers; atrophy of deltoid, arm, forearm, and hand. Muscular movements of left upper extremities fairly well performed; good strength.

Cranial nerves: Refuses to respond to any tests to determine hearing or vision, but evidently hears what is said to him, and in his movements gives no evidence of deafness. Right corner of mouth droops; tongue protrudes straight.

Reflexes: Pupils dilated; margins irregular; left pupil larger; they vary in size but it is impossible to determine whether the variation is due to light or accommodation reflex. Reflexes of right side extremely exaggerated throughout; there is little ankle clonus; Babinski is not obtained, patient holding his toes in flexed position in resisting attempts to elicit reflexes.

Sensations: Reaction to pain stimuli on either side. Evidently some anesthesia on right side, but pressure is apparently very painful. There is considerable spasticity of limbs on right side on passive motion. Too demented to make accurate tests.

The above examination was made on May 6, 1904. On May 20th the record states:

There is almost complete sensory aphasia with word-deafness; some paraphasic circumlocution. Many of his words are very well enunciated but have no meaning. Is apparently unable to recognize objects or their uses.

Brother stated that he was always supposed not to be over bright. Physician’s certificate states that he is epileptic, averaging two attacks per week. On the 15th of May he had a general convulsion; was unconscious for half an hour, and dull and drowsy for two hours afterwards. On the 19th, he had a similar attack in the afternoon, the convulsion lasting a minute, and he was stuporous for an hour.

On November 8th he had a severe epileptic convulsion. His body was curled up to the right. The convulsive seizure lasted for two minutes and was followed by complete unconsciousness for an hour, when the patient roused and appeared as usual in a few minutes. From that time to December 15th he had five epileptic convulsions; he was much more feeble, and unable to help himself as much as formerly.

Nov. 7, 1905: Patient has had occasional convulsions since last note, but none during the last three months. He is confined to bed, has become very much demented, and shows very marked speech defect, so that he is almost unintelligible. He understands only the simplest directions. Legs are considerably contracted and knees are flexed. Arm and hand on the right are paralyzed and show some atrophic changes; partially flexed. Left elbow jerk is very lively. On May 23, 1906 he was reported as having Achilles on right side only, and Babinski on right side. He died January 5, 1907.

The autopsy findings were as follows:

Head: Calvarium of moderate thickness; diploë present; dura slightly adherent over bregmatic region. Longitudinal sinus contains cruor clot. Dura is somewhat thickened and slightly more opaque than normal. Pacchionian granulations, small but fairly numerous. Pia contains throughout a considerable excess of clear serous fluid. The convolutions in general are of good breadth and proportion. There is an atrophic area roughly circular in outline and about 2 cm. in diameter in the posterior part of the right third frontal convolution corresponding to Broca’s area on the opposite hemisphere. The space thus formed is filled with edema held by the pia. On the left side is a similar subpial collection which covers the site of the posterior portions of all of the third frontal convolutions, parts of the lower end of the precentral convolution, and the whole of the first temporal convolution, which have disappeared entirely. The basal vessels show slight changes.

Cerebellum and basal ganglia are grossly normal.

The spinal membranes are negative. The regions of the pyramidal tracts in the cord are firm, project slightly from surface of section, and are china white.

Summary: Here is a picture made up almost purely of Vascular Neurosyphilis, with Secondary Spinal (Pyramidal Tract) Changes. Doubtless the genesis of this picture is allied to that of Case 1 (Alice Morton) and to that of the terminal vascular complications in a tabetic, Case 2 (Francis Garfield).

The absence of meningeal and parenchymatous (i.e., outside the region of necrosis produced by the vascular disease) lesions is characteristic of an important group of neurosyphilitic diseases. It is clear that the case, although one of extensive lesions, is not one of diffuse lesions in the sense of Case 1 (Alice Morton).

The spinal fluid picture in life may nevertheless show (as other cases amply demonstrate) a certain amount of lymphocytosis and possibly plasmocytosis, together with a variety of other changes. Treatment might be expected to keep down these associated changes, although obviously the effects of the necrosis are final and definite. Franz in Washington has succeeded in “reeducating” some of these hemiplegics, employing lower mechanisms of the nervous system.

Vascular neurosyphilis—effects of syphilitic thrombosis of Sylvian artery 10 years before death. (Case 4.)

Case 4. (See previous figure for brain lesion.) Three levels of the spinal cord showing unilateral pyramidal tract sclerosis, 10 years after cerebral thrombosis.

JUVENILE PARETIC NEUROSYPHILIS (“juvenile paresis”). Autopsy.

Case 5. John Lawrence was an under-sized negro, who came under hospital observation when he was 23 years of age. There was some evidence that the patient’s father was a neurosyphilitic although accurate data were out of the question. At all events, John had Hutchinsonian teeth, a forward bowing of the tibiae, and Argyll-Robertson pupils. These findings together with a history of backwardness at school seem to stamp the diagnosis. It seems that there had been a change for the worse from the age of 18, though the boy had been able to sell newspapers and black shoes up to within a year of his arrival at the hospital. During the last months of his life, he showed a general incoördination, with false movements suggesting those of a drunken person. There were numerous tremors, the glance was shifting, and there was a tendency to nystagmus. Some of these phenomena (taking into account that the Hutchinsonian teeth were not entirely typical and there was even at times some doubt as to whether the pupils were actually stiff) led to a question of the diagnosis multiple sclerosis.

There was, however, little doubt that the case was one of juvenile paresis. Among the symptoms found at various times in this case are the following: disorientation for time, place and persons, confusion, with coarsely irrelevant replies to questions, ill-defined and transitory delusions of persecution, auditory, tactile, and visual hallucinations, and defective memory.

Early in life, the patient had had a habit of falling asleep in school hours, and had experienced a number of falls at various times. During an attack of measles he had had a number of spasms, each of which lasted ten minutes or more.

The autopsy showed death to be due to an early bronchial pneumonia. The thymus was persistent, measuring 3 × 2 × .5 cm. The marrow of the femur was red.

There was a moderate degree of sclerosis of the aorta confined to a few plaques in the arch (not a characteristic syphilitic scarring of the aorta). The spleen was small and had a thickened capsule.

The majority of the lesions, however, were in the nervous system, and the following description is taken from the routine hospital records to exemplify the findings in a fairly characteristic case of Juvenile Paresis.

Head: Scalp closely adherent to calvarium. Calvarium heavy without diploë. Dura adherent to calvarium in bregmatic region. Sinuses contain liquid blood. Arachnoidal villi in considerable quantity. Pia mater contains considerable clear fluid and shows diffuse haziness and focal thickenings. The diffuse haziness is almost universal and is best marked over the superior surface of the cerebellum. The focal thickenings are of general distribution over the veins of the sulci on the superior surface of the brim and are heaped up to form considerable linear mounds near the region of the arachnoidal villi. The superior surface of the cerebellum is traversed by similar linear mounds of fibrous tissue running at an angle to the laminæ. There is no notable increase of fibrous tissue at the base.

Brain: Weight 965 grams. The sulcation is roughly symmetrical except in the occipital poles where there is unusually rich and complex but shallow sulcation. The cortical substance is everywhere firmer than normal, but the sulci fail to flare notably. In a few places there is a focal increase of consistence of still greater degree with apparent local hypertrophy (or gliosis with increase of substance). These foci are in the right second temporal gyrus (3 cm. in diameter) and in the left first temporal gyrus (of same size but somewhat less firm) and are of a whitish, waxen appearance, being visible several feet away by reason of their color and apparent encroachment upon the adjacent sulci. The foci are sharply limited by the sulci laterally, but pale out gradually before and behind.

The convolutions of the vertex show another type of lesion. The tissue of the greater part of the vertex resembles that of the flanks and base in being firmer than normal and of a grayish pink color. Behind the fissure of Rolando on the right side and behind the anterior limits of the ascending frontal region on the left side the brain tissue of the vertex becomes suddenly still firmer and of a yellowish gray color. This lesion disappears gradually into the occipital microgyria behind and the gyri gradually lose their yellowish tint. The lesion fades away gradually so that it fails to involve the temporal convolutions.

The cerebral tissue cuts firmly and smoothly. The tissue of the frontal region is a little edematous. The white matter is of a normal appearance. The ependyma of all the ventricles is somewhat sanded. The fourth ventricle is most affected.

The cerebellum is not edematous and is as firm as the normal olivary bodies. The cerebellar hemispheres are symmetrical and of a normal appearance, save that the laminæ are slightly narrower than usual and very compactly set. The color, where not obscured by the haziness of the pia mater, is of a grayish pink somewhat suggestive of freshly tanned shoe leather. The substance cuts smoothly and firmly. The dentate nuclei are unusually firm. The pons is small, but of the usual color. Lower structures normal except the cord which is small and shows curious deviations from the normal markings. The posterior horns and gray commissure are at many levels the only structures to preserve the normal gray appearance, so that the H or butterfly appearance is replaced by a crescent. At these levels, traces of gray matter often stand out in the loci of the anterior horns.

The important anatomical diagnoses in the nervous system are as follows:

Atrophy of cerebrum, 965 grams (there is of course a question whether we are not dealing with a degree of cerebral hypoplasia).

Focal scleroses of cerebrum, suggesting the tuberous scleroses of Bourneville.

Occipital microgyria.

Cerebral and cerebellar gliosis.

Chronic ependymitis.

Gliosis of the gray matter of the spinal cord.

Chronic diffuse and focal leptomeningitis.

The microscopic examination confirmed the diagnosis of paresis. The hypertrophic nodules were of special interest. They were found to be overlain by a characteristic though thin exudate of lymphocytes and plasma cells, together with pigmented cells. The nodules appeared to be supplied with an unusual number of vessels of small calibre, about which were a few lymphocytes. The large vessels and those with well developed adventitiæ were surrounded by more numerous lymphocytes and by more focal accumulations of pigmented cells. The cortex in the middle of a nodule had almost lost its characteristic cortical layering. The cortex was here reduced (specimen from temporal lobe) to about one-quarter of its normal thickness, and was found to be composed largely of expanded neuroglia cells and vascular tissue, with a few nerve elements, small, shrunken, and dark-staining. The destructive process appeared to have borne hardest on the layer of internal large pyramids and the fusiform layer. There was, however, nowhere any evidence of focal necrosis such as ought to characterize a true gumma. The sections stained by the Marchi method failed to show evidence of fatty degeneration within the focus, although there was a marked diffuse accumulation of fatty granulations along the nerve fibres in the underlying white matter. A special study of the cerebellar material was made by one of the authors.^[4] Occasional Purkinje cells showed the characteristic binucleate condition, which has frequently been noted in recent literature.

The cerebellum of this case was perhaps the most markedly diseased of all portions of the nervous system. As noted, the cerebellar tissue was exceedingly firm. How far the notable incoördination of the case (he was observed on staff rounds characteristically curled up in a heap, showing quite an unusual degree of general incoördination) was due to the cerebellar lesions, it is perhaps not possible to say.

Summary: John Lawrence, Juvenile Paretic Neurosyphilis, is a foil to Case 3 (James Dixon), paretic neurosyphilis due to acquired syphilis.

Both showed Cerebral Atrophy, but Lawrence the more markedly because of hypoplasia incidental to the congenital origin of his condition.

Whereas Dixon gave little or no sign of stigmata, Lawrence (besides being under-sized, having suspicious teeth, and showing at autopsy a persistent thymus) showed a Hydromyelia and curious trefoil shape to the spinal cord. Dixon on the other hand had liver lesions and arterial lesions of the leg.

The suggestion of Tuberous Sclerosis in Lawrence is not found in Dixon; but we have not found it elsewhere. Bourneville did not describe tuberous sclerosis as syphilitic.

Binucleate Purkinje cells emphasize the congenital source of the lesions in Lawrence.

Plasmocytosis and Lymphocytosis, Perivascular, and (less marked) Meningeal, are found in both the congenital and the acquired cases, as also parenchymatous changes, both nerve cell losses and gliosis. Both also show granular ependymitis.

It is clear that, over and above the factors of destruction evident in both Lawrence and Dixon, the congenital case, Lawrence exhibits also the effects of arrest (in brief not merely atrophy but also hypoplasia). Early treatment is, therefore, theoretically indicated in the juvenile group, which means early diagnosis. Early diagnosis and treatment are still more to be recommended because these juvenile cases progress often very slowly at first.

FOCAL BASILAR MENINGEAL NEUROSYPHILIS (“syphilitic extraocular palsy,” plus other symptoms). Autopsy.

Case 6. Flora Black, a housewife of 43 years, had been tired out for a year but had been apparently in fair health. She awoke one day with double vision due to a left internal strabismus. The visual difficulty gradually passed away so that five months after the sudden seizure she was apparently quite well again. There was one exception: about three or four months after the attack of diplopia, Mrs. Black had begun to feel a kind of weakness in various parts of the face and there were also fairly definite paresthesiæ. In the sixth month after the initial attack, the patient began to be unable to chew and was fain to support the lower jaw with a bandage to aid in mastication. Deglutition was, however, quite unaffected and there was never any regurgitation of food. There were pains in the face, the forehead and the back of the neck.

Upon physical examination at entrance to a general hospital, no changes in the body at large were discoverable. There was a slight edema of the ankles, otherwise no sign of bodily disease.

Conditions in the head were as follows: The facial lines were (notes by courtesy of Dr. E. W. Taylor) smoothed out; both upper and lower eyelids and the corners of the mouth drooped slightly and more markedly on the left side. There was slight photophobia and considerable lachrymation. The patient was unable to pucker forehead, nose or mouth. The unsupported lower jaw fell and the patient was unable to open the mouth widely. The movements of the tongue were normally performed. Speech was mumbling. Sensations of touch, heat and cold were preserved all over the face except that the left cheek below the level of the mouth yielded a less accurate registration of tactile sensations. A hot test tube did not feel as hot in the lower left cheek as elsewhere. Quinine and sugar could not be tasted over the left half of the tongue in front. Smell and hearing were also diminished on the left side. It appeared that there was a complete paralysis of the 5th and 7th nerves and a partial paralysis of the 8th, 11th and 12th, as well as a defect in smell.

The patient died suddenly, three weeks after admission, running a slight temperature during her stay. The autopsy showed (rather surprisingly) a double ovarian carcinoma with metastases into the retroperitoneal glands. Both kidneys were found to be riddled with nodules of carcinoma. The pelvic veins were thrombosed and there was a complete occlusion of the pulmonary artery. There was a riding embolus in the foramen ovale and there was coronary embolism.

The striking nature of these complications and the interest of the case neurologically would warrant its publication in complete detail. We here present the case with utmost brevity as an example of a Syphilitic Cranial Neuritis by extension from the meninges.

The brain was in general without change but there was a considerable exudate over the entire pontine region which had involved several cranial nerves. The 5th nerves, especially the left, showed gross effects of the inflammatory lesion. There seems to be little or no doubt that this neuritis was of syphilitic origin despite the complication of the case with carcinoma of the ovary and despite the fact that the case was observed and came to autopsy before the modern methods of systematic diagnosis could be applied. It is the best case available to us for the demonstration of a focal cranial nerve lesion of the type characteristic of neurosyphilis. We may well suppose that similar conditions would have been found at various stages in the development of Case 1 (Alice Morton). The pontine region of Case 1 was entirely free from lymphocytic exudate at the time of the autopsy. Possibly the clearing up of the pontine pia mater in Case 1 was a therapeutic effect of the thorough treatment therein used. Whether a case like Mrs. Black’s could be cured (aside from the ovarian carcinoma and its complications) by the institution of vigorous systematic treatment is a matter of doubt. Still, in a general way, these cases of focal syphilitic neuritis are among the most favorable cases for treatment.

Summary: We present the case of Flora Black to emphasize how slight in extent and theoretically curable neurosyphilis may be. We fear that Case 1 (Alice Morton) may present too unrelieved and pessimistic a picture. The extensive vascular lesions and complications of Alice Morton, of Case 2 (Francis Garfield), of Case 4 (James Pierce) arrest attention by the incurability of their residual effects (if we omit modern attempts at reeducation of lower arcs). On the other hand the unrelenting progress to destruction of important parenchymatous structures, as shown in the paretic James Dixon (Case 3) and his juvenile replica John Lawrence (Case 5), as well as in Alice Morton (Case 1) and the tabetic Francis Garfield (Case 2), lead to a certain justifiable pessimism. For it is only the meningeal and fine vascular infiltrations of these cases that we can theoretically hope to combat, probably by destroying the spirochetes in these meningeal and perivascular loci. We seem theoretically less able to stop the progress of the often highly systemic and symmetrical, parenchymatous lesions of the tabetic and paretic group.

The condition in Flora Black is clearly much more hopeful, both being more focal and being almost purely meningeal and therefore accessible to therapy.

The two cases which conclude our general survey of neurosyphilis are also focal cases, one of gumma (Lecompte) and one of focal dural lesion (Wyman).