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THE distinction between general paresis and cerebral syphilis has already been given in the preceding chapter, where also attention has been drawn to the fact that cases have been reported which seem to constitute transition or combination forms.
Cerebral syphilis is not so often met with as general paresis, at least in hospitals for the insane. Thus, of a total of 6265 first admissions to the New York state hospitals during the year ending September, 30, 1914, 774 were cases of general paresis, and but 47 of cerebral syphilis. It is probable, however, that some cases failed to be included in this number having been placed in the cerebral arteriosclerosis group.
Three types of cases are usually distinguished, diffuse meningitic type, gummatous type, and endarteritic type, the distinctions being drawn more or less arbitrarily and, of course, not on the basis of any essential difference in the nature of the pathological process.
Yet the distinctions are valuable not only from a scientific standpoint, but also from that of prognosis, as the three types vary considerably in clinical course, reaction to treatment, and outcome.
1 Twenty-sixth Annual Report of the N. Y. State Hospital Commission, Albany, 1915.
Diffuse meningitic type. — Nearly half of all cases may be said to be of this type. It is apt to occur comparatively early in the course of syphilis, as a rule within five years after the initial lesion. Its onset is usually rapid, the symptoms reaching complete development in two or three weeks. Anatomically it is characterized by a subacute diffuse meningeal inflammation, most marked at the base or even limited to that region, with occasional miliary gummata; the pial blood vessels are the seat of more or less widespread and more or less pronounced endarteritis; the process may sụbside in one area while extending to another, thus producing a peculiarly varying clinical picture.
The symptoms are physical and mental. The physical symptoms, in order of importance, are headache, dizziness, vomiting, convulsions, and evidences of cranial nerve involvement, - amaurosis, ptosis, strabismus, facial neuralgia, hyperæsthesia or anæthesia, facial paralysis, impairment of the sense of smell, and possibly deafness; the pupillary reaction to light and distance may be sluggish or limited in excursion, but the Argyll-Robertson sign is generally absent; a spastic and partly paralytic condition of the lower extremities with increased knee jerks and bilateral or unilateral Babinski sign is often found. The mental symptoms are also very important. “A very characteristic sign of basic syphilitic meningitis is the semi-somnolent, semi-conscious, semi-comatose condition, in which the mental functions are more or less obfuscated rather than obliterated. The patients may present
a lethargic, typhoid, or semi-intoxicated condition, from which they can be temporarily roused — a condition which is, however, frequently combined with a purposeless, hazy motor delirium, not of a purely automatic character. Even in the lesser degrees of obnubilation of consciousness, there are certain criteria of special significance; thus a patient may be roused to more or less correctly answer questions in a slow, drawling, dreamy, sleepy manner. He may even perform complex acts in response to requests or demands, yet be unable to respond to the calls of nature, and he passes urine and fæces in the bed, or evacuates his excreta in the room. Occasionally the patient may shamelessly masturbate. The mind may again become clear and he may regain control, but not infrequently this loss of control over the sphincters persists, and this denotes usually a permanent state of dementia. The dementia of syphilitic brain disease is characterized by being partial and recurring in attacks; it does not alter the character and personality of the individual to the same extent as in the dementia of general paresis. He preserves his autocritical faculties and is conscious of his intellectual deficit, and he is by no means indifferent to his mental and bodily condition. He may suffer with loss of memory, especially of recent events, and his knowledge of time and place may be defective. He is subject to sudden fits of excitation with motor restlessness or of depression with suicidal tendencies.”ı
1 F. W. Mott. Syphilis of the Nervous System. A System of Syphilis, edited by D'Arcy Power and J. K. Murphy, Vol. IV. London, 1910.
Gummatous type. — This type is comparatively infrequent. It is characterized anatomically by the presence of one or more large gummata originating in the meninges and extending into the brain substance. The physical symptoms are apt to be those of brain tumor together with hemianopsia, aphasia, convulsions, hemiplegia, etc., according to the location of the gummata. The mental symptoms are much like those of the diffuse meningitic type.
Endarteritic type. — This is perhaps the commonest type of cerebral syphilis, especially if we take account of the circumstance that many cases are difficult to distinguish from cerebral arteriosclerosis and are often classified as such. The clinical manifestations are, in fact, essentially those of cerebral arteriosclerosis. Even post mortem the differentiation cannot always be made with certainty; the characteristic finding in cerebral syphilis is a proliferative endarteritis accompanied by more or less marked lymphoid and plasma cell infiltration of the adventitial sheaths and, perhaps, patches of similar infiltration in the pia.
Various combinations, forms of the three abovementioned types of cerebral syphilis, are found in practice.
Diagnosis. Cerebral syphilis often has to be differentiated from brain tumor, general paresis, and cerebral arteriosclerosis.
In cases of brain tumor the presence of the cardinal symptoms and focal symptoms and the absence of lymphocytosis in the cerebral-spinal fluid and of the
Wassermann reaction both in the blood and in the fluid will exclude cerebral syphilis.
When the clinical differentiation from general paresis is uncertain, some help may be gained from an examination of the cerebro-spinal fluid; the Wassermann reaction is positive in from 75 to 90% of cases of general paresis and in but 30 or 35% of cases of cerebral syphilis;' in the latter condition it is most apt to be positive in cases of the diffuse meningitic type and negative almost as a rule in the gummatous and endarteritic types; lymphocytosis is almost invariably present in general paresis, the usual finding being from 15 to 50 cells per cubic millimeter, while in cerebral syphilis it is inconstant and extremely variable in degree, being very often slight or absent in the gummatous and endarteritic types and as a rule extremely marked in the diffuse meningitic type — from 100 to 1500 cells or more
per cubic millimeter;2 the typical reaction obtained in the colloidal gold test in cases of general paresis is not apt to be obtained in cerebral syphilis, there being, instead, as a rule, but a slightly marked precipitation in the first one or two tubes, a mere change of color in the next two or three, a more intense reaction again in the next one, two, or three tubes, and no change at all in the remaining ones — the so-called luetic curve which may be represented by the formula, 3321122200.3
1 D. M. Kaplan. Serology of Nervous and Mental Diseases. Philadelphia and London, 1914, p. 191.
2 D. M. Kaplan. Loc. cit., p. 157.
3 Swalm and Mann. The Colloidal Gold Test on Spinal Fluid in Paresis and Other Mental Diseases. N. Y. Med. Journ., Apr. 10, 1915.