Part 42

This favorable result is much influenced by the character of the case, which is sometimes very mild from the outset, and by the thoroughness of the treatment. It is sometimes complicated with iritis, kerato-iritis, cyclitis, posterior choroiditis, secondary glaucoma, etc., in which cases of course the prognosis is most unfavorable. Even in ordinary cases it should be guarded in respect to perfect restoration of function, as clearing of the corneæ may reveal adhesions from iritis or spots of choroiditis disseminata, which could not, of course, have been previously detected. In very mild cases, however, without much evidence of involvement of the other tunics, and which have been submitted to treatment early, it is not uncommon for the cornea to regain almost perfect transparency.

The diagnosis of this condition may generally be made with ease. The ground-glass appearance in the earlier stages and the dull pink or salmon color in the more vascular stage are very characteristic. The vascularity differs from that attending other chronic forms of keratitis, granular lids, etc., in which the vessels are large and superficial, in that in the syphilitic keratitis they are much deeper and very closely interwoven, so that the effect is almost that of an ecchymosis. In other cases both eyes are not so apt to be affected, nor is the tendency to spontaneous cure {300} so marked. The absence of ulceration and the very slight degree of accompanying sclerotic or ciliary congestion are also valuable features.

The conditions which Mr. Hutchinson has known to be most frequently mistaken for it are certain forms of inflammation following small-pox and very superficial ulcers in a healing stage, together with cases of vascular conjunctiva.

There is also said to be some difficulty in distinguishing it from a form of non-syphilitic relapsing cyclitis with corneal opacities and iritis. This, however, is a disease of adults, often limited to one eye, with a strong tendency to relapse, the opacities being more abruptly defined and limited to the region near the circumference.

The chief point of interest, however, in the diagnosis of interstitial keratitis is its association with other symptoms of syphilis, upon which, for the general practitioner at least, the diagnosis will usually depend. Mr. Hutchinson's conclusions, drawn from an analysis of 102 cases of interstitial keratitis, bear strongly upon this point, and are as follows: A large proportion of all cases occur in patients between the ages of eight and fifteen, the disease being comparatively rare in early childhood, and still more so after adult age has been reached. He never saw it begin after the age of twenty-six. The patients presented the physiognomical peculiarities of inherited syphilis (see p. 313). In more than half the cases the previous history, especially as regards infancy, was one of hereditary syphilis, and in many instances there was a clear history of infantile syphilis in brothers or sisters. In half the cases no questions were asked as to the existence of venereal disease in the parents prior to the birth of the child. In 29 out of the other half such disease was freely admitted. All the patients had lost in early life nearly half their brothers and sisters. Omitting miscarriages and premature births, it was found that 77 mothers had borne 547 children, of whom only 284 remained alive--an excessive rate of mortality. It appeared in the large proportion of cases in the eldest children in their respective families--a circumstance to be expected in view of the usual behavior of hereditary syphilis in families. Undoubted syphilitic lesions, such as nodes, ulceration of the palate, etc., are not infrequently associated with the keratitis.

Examination of large numbers of cases has, I believe, established the general accuracy of these statements; and although there is still much difference of opinion as to the exact relation between syphilis and this form of keratitis--whether, for example, it is a symptom of syphilis itself or of a cachexia frequently produced by syphilis, but often by other systemic diseases affecting nutrition--yet, on the whole, so far as I am able to judge of the question outside of its purely technical and ophthalmological relations, I think the weight of modern authority is chiefly on the side of a distinct and practically invariable relation of cause and effect between inherited syphilis and the corneal inflammation.

{301} Mr. James Dixon, for example, proposes to call the disease syphilitic keratitis, and says it is met with exclusively as a sequel of an inherited taint. He adds: "We may meet with some syphilitic keratitis in patients with healthy physiognomy and deformed teeth, or, still more rarely, in those with faultless teeth and the syphilitic cast of features; but to find the true form of keratitis in connection with both good teeth and good complexion is, I think, next to impossible." Many other ophthalmologists express themselves to the same effect more or less strongly. Nettleship, Noyes, Förster, Macnamara, De Wecker, and Carter may be mentioned as having ranged themselves upon this side. On the other hand we have Schweiger, Maunther, Sæmisch, Soelberg Wells, and others who are not convinced that syphilis is the sole nor even, in the opinion of some of them, the principal cause of this disease.

Probably the conclusions of Jullien most nearly express the views of those who do not accept in its entirety the syphilitic theory of the disease. He concludes--(1st) that interstitial keratitis coincides frequently, but not invariably, with syphilitic derangement of the dental apparatus; (2) that it is associated with a feeble constitution and with malnutrition, and is thus produced indirectly by scrofula, rheumatism, or syphilis, each of which by its debilitating influence may give rise to a diathesis which favors such morbid developments.

As a specimen of the evidence which is produced by observers other than Mr. Hutchinson--who, as he himself observes, may be suspected of "that bias which almost necessarily warps more or less the judgment of one who supposes himself to have noted something new"--the statistics of Förster, who apparently takes an unbiassed view of the question, may be noted: In a total of 214 cases of interstitial keratitis evidence of hereditary syphilis other than the corneal disease was noted in 146 (68 per cent.). This number was made up as follows: a, evidence of syphilis in parent, 17; b, evidence of hereditary syphilis in other members of the family, 14; c, evidence in the patient other than keratitis, 115; characteristic teeth, 69; evidences in physiognomy, bones, skin, palate, or choroid, 46. In the remaining 68 cases (32 per cent.), though inheritance of syphilis was not proved, there was, almost without exception, strong suspicion of that disease apart from the keratitis.

The condition of the permanent central upper incisors was noted in 138 of the cases; of these they were typical in 73 (53 per cent.); suspicious in 34 (24 per cent.); normal in 31 (23 per cent.). In 15 cases {302} the permanent teeth had not been cut, and in the remaining 61 the state of the teeth was not recorded.

This seems to me such direct and such unequivocal clinical evidence that it is safe to say, as of the question of syphilitic teeth, that the burden of disproof rests with the doubters, and that we may venture the assertion that interstitial, diffuse, or parenchymatous keratitis is a symptom of inherited syphilis, and that the unmistakable presence of the former disease is sufficient proof of the existence of the latter.

Syphilis of the Nerve-Centres and Nerves.--Until a comparatively recent period our only guide to the course and progress of the nerve diseases of inherited syphilis was to be found in analogy. We knew, for instance, that in acquired syphilis three forms of cerebral disease could be recognized in a general way--one characterized by sudden attack of paralysis, in which the lesion was usually thrombosis from specific endo-arteritis; one in which the symptoms of brain tumor were present, and in which gummata were the cause of the difficulty; and one in which pain, headache, and various functional or convulsive disturbances--chorea, epilepsy, paralysis of single nerves, etc.--were the customary phenomena, and in which periosteal, meningeal, or neuroglial thickenings constituted the pathological basis. The last two are often intermingled both symptomatically and histologically.

Heubner divides cerebral syphilis into three groups, two of which very closely resemble those I have described. In one, however, he includes both the general physical disturbances, incomplete paralysis, and final coma characteristic of tumor and the epileptiform attacks so often due to peripheral or meningeal irritation. This combination is explained by the results of his autopsies, which disclosed in 26 cases in which these symptoms were conjoined a gummous growth in the pia mater of the convexity of one of the cerebral hemispheres, either limited and superficial or involving more or less of the cortex and forming a distinct tumor. The epileptiform attacks were present in 19 out of these 26 cases, while in 20 other cases where the growth was limited to the white substance at the base of the brain they were present only twice. This second form is the apoplectic, followed by general hemiplegia, and depending on disease of the cerebral arteries. His third division is a very ill-defined one, depends much for its limitations upon subjective symptoms, and is of no special interest as applied to the subject of inherited syphilis.

Althaus also makes three divisions, two of which are as follows: (1) Cerebral tumor--a gumma either hard or soft. There are then nocturnal headache, sleeplessness, epileptiform attacks, the various phenomena produced by involvement of the cerebral nerves, etc. (2) Disease of the arteries, apoplexy, or softening, followed by hemiplegia.

Hutchinson makes a similar division of lesions and symptoms, and the observations of Jaksch, Wilks, and Hughlings Jackson more or less closely coincide with this general classification.

Now, in spite of certain striking differences--more apparent than real, however--between inherited and acquired syphilis as regards cause, {303} duration of stages, etc., the essential pathological changes are the same. When syphilis in its later periods attacks the brain or spinal cord or nerve-trunks or vessels of a foetus, it proceeds just as in the adult, the same characteristic accumulation of cells taking place and setting up an arteritis or a meningitis, thickening the sheaths of nerves, or constituting a pericranial node or a gumma according to their number and their situation. We would accordingly expect to find in subjects of inherited syphilis manifestations closely allied to those observed in the adult; and the observations of Barlow, Graefe, Jackson, Heubner, Dowse, and Hutchinson, though comparatively few in number, have already demonstrated the correctness of this supposition.

We find, thus, that in these patients meningitis, growths, and arterial disease constitute the three clinical divisions of the disease which have thus far been distinctly differentiated, and the reported cases, with or without autopsy, fall naturally into these classes.

These cases are naturally few, and to make them absolutely reliable it is necessary to have unmistakable evidence of hereditary syphilis in some other form and the demonstration of syphilitic lesions at an autopsy. The case of Dowse, however, includes these requirements. A child twelve years of age, of syphilitic parents, with a history of coryza, sore eyes, and a tubercular syphilide, was attacked with epilepsy, diplopia, facial paralysis, etc., and finally died. At the autopsy three gummatous growths of the surface of the brain were found, and the vessels of the base were found to have undergone the special changes described by Heubner. Their lumen was in some places nearly occluded by an accumulation of spindle-shaped cells between the tunica fenestra and the epithelial lining; and interspersed with them, but particularly in the muscular and adventitious coats, were to be seen enormous quantities of round cells which in many parts seemed actually to replace the normal structures. Dowse's other cases are not at all conclusive in their clinical histories; even the diagnosis was not established by autopsy.

Barlow's two cases were both very young children, and are extremely convincing. A child four months old, with snuffles, serpiginous ulcers, etc., and with a syphilitic father, had epileptiform attacks, followed by laryngismus, carpo-pedal contraction, and changes in the choroid. She died aged about fifteen months, and the autopsy disclosed thickening of the pia mater, evidently not tubercular, and changes in the arteries, which in the gradual narrowing of the lumen of the vessel, the absence of ulceration or disintegration or calcification, and the continuity and extent of the cell-proliferation are as different as possible from ordinary atheroma, but correspond precisely with the description of Heubner's cases, which were undoubtedly the subjects of acquired syphilis.

In the second case the symptoms were associated chiefly with the cranial nerves. These were nystagmus, paresis of facial muscles, laryngeal spasms, etc. He died at the age of fifteen months. The vessels of the base were extensively diseased as in the other case, and the fourth, {304} fifth, sixth, seventh, and eighth pairs of nerves were smaller by a new growth of a gummatous nature which had produced almost entire atrophy of the nerve-cylinders. There were cicatrices of the liver and spleen. Cases in which the diagnosis rested upon the history, upon the co-existence of undoubted syphilitic symptoms, and upon improvement under specific treatment are by no means rare.

Berkely Hill reports a typical case of syphilitic epilepsy. A girl aged nine was the first child of the family that had lived, two having previously been born dead. She presented characteristic teeth, traces of choroiditis, and, while under treatment, suffered from both keratitis and iritis. Her first fit was when she was four years of age, and the attacks had recurred frequently since that time. The convulsion was confined to the left side. The left arm was very weak, the weakness having come on gradually, and being especially great in the extensors of the wrist. Under specific treatment the attacks ceased entirely. In this case there was certainly organic disease, probably a gumma on the surface of the right hemisphere.

Other cases reported by the same author, numerous instances of nervous troubles in inherited syphilis reported by Hughlings Jackson, Fournier, Henoch, and Hutchinson, warrant the statement, then, that the nervous diseases of inherited syphilis fall into the same general category as those of the acquired disease; that they may appear at any age, from three or four months to that of puberty; that they depend for their production upon a cell-proliferation which, according to its locality, results in the development of new growths, the production of localized meningeal inflammations, or the obstruction and inflammation of arteries; and that the symptoms are those usually associated with such pathological changes, the diagnosis of syphilis depending chiefly on the history and the presence of other specific phenomena.

The prognosis is more unfavorable than in similar cases in acquired syphilis, the meningeal and vascular lesions being the most frequent and showing themselves very obstinate even under careful treatment. Spinal troubles, chorea, and idiocy have been attributed to inherited syphilis, but must occur with great rarity.

Hill reports a case of a child aged five years who had been hemiplegic since he was two and a half years old, and who developed paralysis of the flexors of the ankle in each leg.

Keyes reports a case of a boy five years of age, with nodes on tibiæ and other signs of inherited syphilis, who had two attacks of paraplegia.

Hill reports a case of imbecility associated with inherited syphilis, but there is no evidence that it was other than a coincidence.

Fletcher Beach found not more than 1 per cent. of syphilitic children in the Dareult Asylum, and Mr. Mercier could only trace syphilis in 5 out of 220 female idiots, probably imbecile from birth.

Hughlings Jackson only found 1 case of inherited syphilis among 80 {305} cases of chorea. The most carefully recorded cases of the latter affection associated with hereditary syphilis are two reported by Alison.