Early Involvement

"Recent developments have shown that the occurrence of syphilis of the central nervous system, more particularly of the meninges, is much more common in the secondary stage than has been generally recognized, and that it may occur even earlier than the secondary skin manifestations. Various observers have shown by lumbar puncture that in about 35% of all untreated cases there is an increased cell count and an increased globulin content of the cerebro-spinal fluid, an indication of syphilitic involvement of the meninges. In the early stages the spinal fluid Wassermann is usually negative. Early involvement of the central nervous system is frequently betrayed by certain complaints of the patient - headache, neuralgia, dizziness, slight deafness, slight paralysis of one of the cranial nerves." 1

"I believe that practically all cases of neuro-syphilis originate in the early stages of the disease. This belief is based upon the results of several hundred serological examinations made in the first year of the infection. In other words the number of cases of neuro-syphilis which develop is not greater than the number of patients which show positive serological findings at this time. As a corollary to this statement, I further believe that if, before a patient is discharged as cured, his spinal fluid is found to be normal, he will never develop paresis or other forms of neuro-syphilis. There is, of course, no proof as yet available that this hypothesis is correct. It will require a good many years of observation before a definite statement of this kind can be made. It is, however, a good working hypothesis and up to this time I have never seen a case of neuro-syphilis develop in a patient with negative spinal fluid after the first or second year of his infection."

1 H. H. Young. Manual of Military Urology. Published for the American Expeditionary Forces by the American Red Cross, Paris. 1919.

"When involvement of the fluid does occur in the secondary period of the disease it is much more amenable to treatment than in old cases where the infection has occurred many years previously. I believe that neuro-syphilis is due to a special strain of spirochete which may have invaded the fluid during the most intensive administration of mercury and salvarsan. In these cases it is only possible to reach the infection by intraspinal treatment. I have had a number of cases of very intense meningitis which developed a few months after infection shortly after the prolonged use of salvarsan and mercury. These patients are now entirely cured both clinically and serologically by intraspinal treatment." 1

Meningitic Type

This type 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 subside 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, hyperesthesia or ansethesia, 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, semiconscious, 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 faeces 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.

1 J. H. Fordyce, in a personal communication, dated May 8, 1918.

He is subject to sudden fits of excitation with motor restlessness or of depression with suicidal tendencies." l

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 combination-forms of the three above-mentioned types of cerebral syphilis are found in practice.

Diagnosis

Cerebral syphilis often has to be differentiated from brain tumor, general paralysis, and cerebral arteriosclerosis.

In cases of brain tumor the presence of the cardinal symptoms and focal symptoms and the absence of lymphocytosis in the cerebro-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 paralysis 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 paralysis and in but 30 or 35% of cases of cerebral syphilis;1 in the latter condition it is most apt to be positive in cases of the meningitic type and negative almost as a rule in the gummatous and endarteritic types; lymphocytosis is almost invariably present in general paralysis, 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 meningitic type - from 100 to 1500 cells or more per cubic millimeter;2 the typical reaction obtained in the colloidal gold test in cashes of general paralysis 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 - 3321122200.3

1F. W. Mott. Syphilis of the Nervous System. A System of Syphilis, edited by D'Arcy Power and J. K. Murphy, Vol. IV. London. 1910.

The test of treatment is of value in many cases, improvement or recovery under salvarsan or mercury and iodides with reduction or disappearance of the lymphocytosis indicating cerebral syphilis and not general paralysis.

In cerebral arteriosclerosis the findings in the cerebrospinal fluid are negative, so that a difficulty in differentiation arises only in connection with those cases of the endarteritic type of cerebral syphilis in which the findings are likewise negative, and in such cases, as already stated, the differentiation cannot always be made with certainty even post mortem. A history of syphilitic infection will, naturally, turn the probability toward cerebral syphilis. The age of the patient may help in the differentiation: cases occurring in persons under forty-five are almost surely syphilitic; in persons between forty-five and sixty the probability is still strongly in favor of syphilis; after sixty this probability diminishes with advancing senility.

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.

Prognosis

Cerebral syphilis is a grave affection; untreated cases progress more or less rapidly with tissue destruction and often a fatal termination. Treatment, however, if instituted early may result in a quick and perfect cure; the most favorable cases from this point of view are those of the meningitic type; cases of the gummatous type are often stubbornly resistant to treatment; in most cases of the endarteritic type recovery cannot be expected owing to the tissue destruction which occurs quickly, but much improvement may result from prompt and vigorous treatment.