Chapter LXVI: Introduction: Notwithstanding the numerous measurings and weighings of (2)

The general grounds of diagnosis have been sufficiently mapped out in the last section, but some reiteration may be allowable. After the exclusion of other non-specific disease, headache occurring with any form of ocular palsy or with a history of attack of partial monoplegia or hemiplegia, vertigo, petit mal, epileptoid convulsions, or disturbances of consciousness, or attacks of unilateral or localized spasms, should lead to the practical therapeutic test. Ocular palsies, epileptic forms of attacks occurring after thirty years of age, morbid somnolence, even when existing alone, are sufficient to put the practitioner upon his guard. It is sometimes of vital importance that the nature of the cephalalgia shall be recognized before the coming on of more serious symptoms; any apparent causelessness, severity, and persistency should arouse suspicion, to be much increased by a tendency to nocturnal exacerbations or by the occurrence of mental disturbance or of giddiness at the crises of the paroxysms. Not rarely there are very early in these cases curious, almost indefinable, disturbances of cerebral functions, which may be easily overlooked, such as temporary and partial failures of memory, word-stumbling, fleeting feelings of numbness or weakness, alterations of disposition. In the absence of hysteria an indefinite and apparently disconnected series of nerve accidents is of very urgent import. To use the words of Hughlings-Jackson, “A random association or a random succession of {1013} nervous symptoms is very strong warrant for a diagnosis of a syphilitic disease of the nervous system.” Cerebral syphilis occurring in an hysterical subject may be readily overlooked until fatal mischief is done. When any paralysis occurs a study of the reflexes may sometimes lead to a correct diagnosis. Thus in a hemiplegia the reflex on the affected side in cerebral syphilis is very frequently exaggerated, whilst in hysteria the reflexes are usually alike on both sides. When both motion and sensation are disturbed in an organic hemiplegia, the anæsthesia and motor paralysis occur on the same side of the body, whilst in hysteria they are usually on opposite sides.

In all cases of doubtful diagnosis the so-called therapeutic test should be employed, and if sixty grains of iodide of potassium per day fail to produce iodism, for all practical purposes the person may be considered to be a syphilitic. No less an authority than Seguin has denied the validity of this, but I believe, myself, that some of his reported cases were suffering from unsuspected syphilis. I do not deny that there are rare individuals who, although untainted, can resist the action of iodide, but in ten years' practice in large hospitals, embracing probably some thousands of cases, I have not met with more than one or two instances which I believed to be of such character. Of course in making these statements I leave out of sight persons who have by long custom become accustomed to the use of the iodide, for although in most cases such use begets increase of susceptibility, the contrary sometimes occurs. Of course the physician who should publicly assert that a patient who did not respond to the iodide had syphilis would be a great fool, but in my opinion the physician who did not act upon such a basis would be even more culpable.

PROGNOSIS.—Cerebral meningeal syphilis varies so greatly and so unexpectedly in its course that it is very difficult to establish rules for predicting the future in any given case. The general laws of prognosis in brain disease hold to some extent, but may always be favorably modified, and patients apparently at the point of death will frequently recover under treatment. The prognosis is not, however, as absolutely favorable as is sometimes believed, and especially should patients be warned of the probable recurrence of the affection even when the symptoms have entirely disappeared. The only safety after the restoration of health consists in an immediate re-treatment upon the recurrence of the slightest symptom. The occurrence of a complete, sudden hemiplegia or monoplegia is sufficient to render probable the existence of a clot, which must be subject to the same laws as though not secondary to a specific lesion. If a rapid decided rise of temperature occur in an apoplectic or epileptic attack, the prognosis becomes very grave. An epileptic paroxysm very rarely ends fatally, although it has done so in two of my cases.

The prognosis in gummatous cerebral syphilis should always be guardedly favorable. In the great majority of cases a more or less incomplete recovery occurs under appropriate treatment, and I have seen repeatedly patients who were unconscious, with urinary and fecal incontinence, and apparently dying, recover. Nevertheless, so long as there is any particle of gummatous inflammation in the membrane the patient is liable to sudden congestions of the brain, which may prove rapidly fatal, or he may die in a brief epileptic fit. On the one hand there is an {1014} element of uncertainty in the most favorable case, and on the other so long as there is life a positively hopeless prognosis is not justifiable.

PATHOLOGY.—Gummatous inflammation of the brain probably always has its starting-point in the brain-membranes, although it may be situated within the brain: thus, I have seen the gummatous tumors spring from the velum interpositum in the lateral ventricle. The disease most usually attacks the base of the brain, and is especially found in the neighborhood of the pons Varolii and the optic tract. It may, however, locate itself upon the vault of the cranium, and in my experience has seemed to prefer the anterior or motor regions. The mass may be well defined and roundish, but more usually it is spread out, irregular in shape, and more or less confluent with the substance of the brain beneath it. It varies in size from a line to several inches in length, and when small is prone to be multiple. The only lesion which it resembles in gross appearance is tubercle, from which it sometimes cannot be certainly distinguished without microscopic examination.

The large gummata have not rarely two distinct zones, the inner one of which is drier, somewhat yellowish in color, opaque, and resembles the region of caseous degeneration in the tubercle. The outer zone is more pinkish and more vascular, and is semi-translucent.

On microscopic examination the most characteristic structures are small cells, such as are found in gummatous tumors in other portions of the body. These cells are most abundant in the inner zone, which, indeed, may be entirely composed of them. In the centre of the tumor they are more or less granular and atrophied; in some cases the caseous degeneration has progressed so far that the centre of the gumma consists of minute acicular crystals of fat. In the external or peripheral zone of the tumor the mass may pass imperceptibly into the normal nerve tissue, and under these circumstances it is that it contains the spider-shaped cells or stellate bodies described by Jastrowitch, and especially commented upon by Charcot and Gombault and by Coyne. These are large cells containing an exaggerated nucleus and a granular protoplasm, which continues into multiple, branching, rigid, refracting prolongations, which prolongations are scarcely stained by carmine. Alongside of these cells other largish cells are often found without prolongations, but furnished with oval nuclei and granular protoplasm. Amongst these cells will be seen the true gummatous cells, as well as the more or less altered neuroglia and nerve-elements. In the perivascular lymphatic sheaths in the outer part of the gumma is usually a great abundance of small cells. The spider-shaped cells are probably hypertrophied normal cells of the neuroglia, and have been considered by Charcot and Gombault as characteristic of syphilitic gummata of the brain. In a solitary gumma, however, of considerable size from the neighborhood of the cerebellum, studied by Coyne and Peltier, there were no stellated cells. Coyne considers that their presence is due to their previous existence in the normal state of the regions affected by the gumma. Exactly what becomes of syphilitic gumma of the brain in cases of recovery it is difficult to determine. It is certain that they become softened and disappear more or less completely, and it is probable that the cicatrices or the small peripheral cysts which are not rarely found in the surfaces of the brain are sometimes remnants of gummatous tumors. In a number of cases collected by Gros and {1015} Lancereaux there were small areas of softened tissue or small calcareous and caseous masses or cerebral lacunæ corresponding to the cicatrices of softening or imperfect cysts, coincident with evidences of syphilis elsewhere. V. Cornil also states that he has found small areas of softening with well-established syphilitic lesions of the dura mater and cranium, but believes that the lacunæ or cysts depend rather upon chronic syphilitic lesions of cerebral arteries than upon gummatous inflammation.

When a gummatous tumor comes in contact with an artery, the latter is usually compressed and its walls undergo degeneration. The specific arteritis may pass beyond the limit of the syphilome and extend along the arterial wall. Not rarely there is under these circumstances a thrombus, and if the artery be a large one secondary softening of its distributive brain-area occurs.

TREATMENT.—The treatment of cerebral syphilis is best studied under two heads: First, the treatment of the accidents which occur in the course of the disease; second, the general treatment of the disease itself.

It must be remembered that in the great majority of cases in which death occurs in properly-treated cerebral syphilis the fatal result is produced by an exacerbation—or, as I have termed it, an accident—of the disease. Under these circumstances the treatment should be that which is adapted to the relief of the same acute affection when dependent upon other than specific cause. In a large proportion of cases the acute outbreak takes the form either of a meningitis or else of a brain congestion. In either instance when the symptoms are severe free bleeding should be at once resorted to. The amount of blood taken is of course to be proportionate to the severity of the symptoms and the strength of the patient. I have seen life saved by the abstraction of about a quart of blood, whilst in other cases a few ounces suffice. Care must be, of course, taken not to mistake a simple epileptic fit for a severe cerebral attack; but when this fit has been preceded by severe headache and is accompanied by stupor, with marked disturbance of the respiration, measures for immediate relief are usually required; and if the convulsions be perpetually repeated or if there be violent delirious excitement, the symptoms may be considered as very urgent. In taking blood the orifice should be large, so as to favor a rapid flow, and the bleeding be continued until a distinct impression is made upon the pulse. In some cases which I have seen in which the action of the heart continued to be violent after as much blood as was deemed prudent had been taken, good results were obtained by the hypodermic injection of three drops of the tincture of aconite-root every half hour until the reduction of the pulse and the free sweating indicated that the system was coming under the influence of the cardiac sedative.

Of course, I do not mean to encourage the improper or too free use of the lancet in these cases, but in the few fatal cases which I have seen I have almost invariably regretted that blood had not been taken at once very freely at the beginning of the acute attack. In most of these cases the symptoms had progressed too far for good to be achieved before I reached the patient. After venesection, or in feeble cases as a substitute for it, the usual measures of relief in cerebral congestion should be instituted. I shall not occupy space with a discussion of these {1016} measures, as they are in no way different from those to be employed in cases not syphilitic.

The most important part of the treatment of cerebral syphilis itself is antisyphilitic, and the practitioner is at once forced to select between the iodide of potassium and the mercurial preparations. In such choice it must be remembered that even a very small amount of syphilitic deposit in the brain may at any time cause a sudden congestion or other acute attack, and is therefore a very dangerous lesion. I have seen a cerebral syphilis which was manifested only by an epileptic attack occurring once in many months, and in which after death the affected membrane was found to be not larger than a quarter of a dollar, and the deposit not more than an eighth of an inch in thickness, suddenly produce a rapidly fatal congestion; and I have known a case fast progressing toward recovery suddenly ended by the too long continuance of the arrest of respiration during an epileptic fit. I have, myself, no doubt of the superiority of the mercurials over the iodide of potassium as a means of producing absorption of gummatous exudates; and as these exudates in the brain are so very dangerous, a mercurial course should in the majority of cases of cerebral syphilis be instituted so soon as the patient comes under the practitioner's care. When, however, there is a history of a recent prolonged free use of the mercurial, or when there is marked specific cachexia, the iodide should be chosen. Cachexia is, however, a distinctly rare condition in cerebral syphilis, the disease usually developing in those who have long had apparent immunity from the constitutional disorder. In my opinion the best preparation of the mercurial for internal use is calomel. It should be given in small doses, one-quarter of one grain every two hours, guarded with opium and astringents, so as to prevent as far as possible disturbance of the bowels, and should be continued until soreness of the teeth, sponginess of the gums, or other evidences of commencing ptyalism are induced. After this the dose of the mercurial should be so reduced as simply to maintain the slight impression which has been created, and the patient should be kept under the mercurial influence for some weeks.

A very effective method of using the mercury is by inunction, and where the surroundings of the patient are suitable the mercurial ointment may be substituted for the calomel. It should be applied regularly, according to the method laid down in my treatise on therapeutics. I have sometimes gained advantage by practising the mercurial unction and at the same time giving large doses of iodide of potassium internally.

After a mercurial course the iodide of potassium should always be exhibited freely, the object being not only to overcome the natural disease, but also to bring about the complete elimination of the mercury from the system. There is no use in giving the iodide in small doses; at least a drachm and a half should be administered in the twenty-four hours, and my own custom has been to increase this to three drachms unless evidences of iodism are produced. The compound syrup of sarsaparilla covers the disagreeable taste of the iodide of potassium better than any other substance of which I have knowledge. Moreover, I am well convinced that there is some truth in the old belief that the so-called “Woods” are of value in the treatment of chronic syphilis. {1017} I have seen cases in which both the iodide of potassium and the mercurials had failed to bring about the desired relief, but in which the same alteratives, when given along with the “Woods,” rapidly produced favorable results. The old-fashioned Zittmann's decoction, made according to the formula of the United States Dispensatory, may be occasionally used with very excellent effect. But I have gradually come into the habit of substituting a mixture of the compound fluid extract and the compound syrup of sarsaparilla in equal proportions. The syrup itself is too feeble to have any influence upon the system, but is here employed on account of its flavor. A favorite method of administration is to furnish the patient with two bottles—one containing a watery solution of the iodide of potassium of such strength that two drops represent one grain of the drug, and the other the sarsaparilla mixture above mentioned. From one to two drachms of the solution of the iodide may be administered in a tablespoonful of the sarsaparilla well diluted after meals. When the patient has been previously mercurialized, or there is any doubt as to the propriety of using mercurials, corrosive sublimate in small doses may be added to the solution of the iodide, so that one-tenth to one-fifteenth of a grain shall be given in each dose. I have never seen especial advantage obtained by the use of the iodides of mercury. They are no doubt effective, but are not superior to the simpler forms of the drug.

Syphilitic Disease of the Brain-Cortex.

The psychical symptoms which are produced by syphilis are often very pronounced in cases in which the paralysis, headache, epilepsy, and other palpable manifestations show the presence of gross brain lesions. In the study of syphilitic disease of the brain-membranes sufficient has been said in regard to these psychical disturbances, but the problem which now offers itself for solution is as to the existence or non-existence of syphilitic insanity—_i.e._ of an insanity produced by specific contagion without the obvious presence of gummatous disease of the brain-membranes. Very few alienists recognize the existence of a distinct affection entitled to be called syphilitic insanity, and there are some who deny that insanity is ever directly caused by syphilis. It is certain that insanity often occurs in the syphilitic, but syphilis is abundantly joined with alcoholism, poverty, mental distress, physical ruin, and various depressing emotions and conditions which are well known to be active exciting causes of mental disorder. It may well be that syphilis is in such way an indirect cause of an insanity which under the circumstances could not be properly styled syphilitic.

If there be disease of the brain-cortex produced directly by syphilis, of course such disease must give rise to mental disorders; and if the lesion be so situated as to affect the psychic and avoid the motor regions of the brain, it will produce mental disorder without paralysis—_i.e._ an insanity; again, if such brain disease be widespread, involving the whole cortex, it will cause a progressive mental disorder, accompanied by gradual loss of power in all parts of the body, and ending in dementia with general paralysis; or, in other words, it will produce an affection {1018} more or less closely resembling the so-called general paralysis of the insane, or dementia paralytica.

As a man having syphilis may have a disease which is not directly due to the syphilis, when a syphilitic person has any disorder there is only one positive way of determining during life how far said disorder is specific—namely, by studying its amenability to antisyphilitic treatment. In approaching the question whether a lesion found after death is specific or not, of course such a therapeutic test as that just given is inapplicable. We can only study as to the coexistence of the lesion in consideration with other lesions known to be specific. Such coexistence of course does not absolutely prove the specific nature of a nutritive change, but renders such nature exceedingly probable.

What has just been said foreshadows the method in which the subject in hand is to be here examined, and the present article naturally divides itself into two sections—the first considering the coexistence of anatomical alterations occurring in the cerebral substance with syphilitic affections of the brain-membranes or blood-vessels, the second being a clinical study of syphilitic insanity.

In looking over the literature of the subject I have found the following cases in which a cerebral sclerotic affection coincided with a gummatous disease of the membrane. Gros and Lancereaux[60] report a case having a clear syphilitic history in which the dura mater was adherent to the skull. The pia mater was not adherent. Beneath, upon the vault of the brain, was a gelatinous exudation. The upper cerebral substance was indurated, and pronounced by Robin after microscopic examination to be sclerosed. At the base of the brain there were atheromatous arteries and spots of marked softening.

[Footnote 60: _Affec. Nerv. Syphilis_, 1861, p. 245.]

Jos. J. Brown[61] reports a case in which the symptoms were melancholia, excessive irritability, violent outbursts of temper, very positive delusions, disordered gait, ending in dementia. At the autopsy, which was very exhaustive, extensive syphilitic disease of the vessels of the brain and spinal cord was found. The pia mater was not adherent to the brain. The convolutions, particularly of the frontal and parietal lobes, were atrophied, with very wide sulci filled with bloody serum. The neuroglia of these convolutions was much increased, and “appeared to be more molecular than normal, the cells were degenerated, and in many places had disappeared, their places being only occupied by some granules.” These changes were most marked in the frontal convolutions.

[Footnote 61: _Journ. Ment. Sci._, July, 1875, p. 271.]

H. Schule reports[62] a very carefully and meritoriously studied case. The symptoms during life exactly simulated those of dementia paralytica. The affection commenced with an entire change in the disposition of the patient; from being taciturn, quiet, and very parsimonious, he became very excited, restless, and desiring continuously to buy in the shops. Then failure of memory, marked sense of well-being, carelessness and indifference for the future, developed consentaneously with failure of the power of walking, trembling of the hands, inequality of the pupils, and hesitating speech. There was next a period of melancholy, which was in time followed by continuous failure of mental and motor powers, and {1019} very pronounced delirium of grandeur, ending in complete dementia. Death finally occurred from universal palsy, with progressive increase of the motor symptoms. At the autopsy characteristic syphilitic lesions were found in the skull, dura mater, larynx, liver, intestines, and testicles. The brain presented the macroscopic and microscopic characters of sclerosis and atrophy; the neuroglia was much increased, full of numerous nuclei, the ganglion-cells destroyed. The vessels were very much diseased, some reduced to cords; their walls were greatly thickened, and full of long spindle-shaped cells, sometimes also containing fatty granules.

[Footnote 62: _Allgem. Zeitschrift f. Psychiatrie_, xxviii. 171, 172.]