As a rule, especially in cases which are recognized early and treated actively, the prognosis is good. Iodide of potassium should be used in combination with mercury.

Syphilis of the Teeth.—Syphilis of the teeth has its chief interest to the general practitioner from its very important bearing on diagnosis. As manifesting itself at an age when the child is not apt to present the active and unmistakable cutaneous and mucous lesions of the disease, and when, consequently, its recognition is often extremely difficult, this diagnostic importance is greatly increased.

The teeth of the first dentition, although exhibiting the usual signs of interference with nutrition in their irregular development, opaque and chalky enamel deficient in quantity and unevenly distributed, soft and friable dentine, incongruity of size individually and relatively, and proneness to decay, do not often display any distinctive evidence of syphilis. The same conditions may, and often do, depend on other causes, and are commonly associated with various cachexiæ—the strumous, gouty, rheumatic, rachitic, etc.—and even with other slighter ailments tending to produce imperfect assimilation and malnutrition.

In the permanent teeth, likewise, the same condition may be due to the same causes. Stomatitis, however produced—by mercury, by gastro-intestinal derangements, by local irritation of any kind—is apt to result in imperfectly organized dental structures. Mercurial teeth, for example, are usually irregularly aligned, horizontally seamed, honeycombed, craggy, malformed, of an unhealthy dirty yellow color, separated too widely, and deficient in enamel.¹²⁴ The diseases of childhood, especially the eruptive fevers, eclampsia, typhoid fever, etc., by temporarily arresting or greatly interfering with nutrition during the developmental period of the teeth, often cause horizontal furrows across their crowns, which are, of course, persistent throughout life, and mark indelibly the influence of such disorders on all the formative processes.

¹²⁴ The latter defect is particularly noticeable on the cusps of the sixth-year molars. (See note on "Syphilis of the Teeth," by Dr. James W. White, in Am. ed. of Cornil, pp. 287-290.) The discussion as to the effect of mercury in producing the condition of the teeth known as honeycombed is still going on, but the evidence seems to point clearly to a direct connection with the administration of mercury in infancy, either for syphilis or in excessive doses as a purge, or in some of the teething powders, which often consist of calomel and opium. Mr. Hutchinson, at a meeting of the Odontological Society (see Proceedings for 1877, p. 249), gave an interesting description of the way in which the supposition was arrived at. Lamellar cataract is a disease which affects the eyes of children who have suffered from convulsions: it was noticed that in cases of that form of catarrh there were also honeycombed teeth, and it was thought that the convulsions, the cataract, and the honeycombed teeth were all due to the same unknown cause. At last a few exceptions were found—patients with cataract, but with good teeth, and then some who had had convulsions only, and yet had honeycombed teeth; lastly, it was noticed that most of the patients had been treated with mercury. So it came to be recognized that the honeycombed teeth were only accidentally associated with the cataract, and that they were, in fact, the result of the mercury which had been given to cure the convulsions. The same gentleman figures a case of this disease in his Illustrations of Clinical Surgery (London, 1875), and thus describes it (p. 55): "The present state of his permanent teeth is so characteristic as to deserve more detailed description. The change about to be mentioned affects all the incisors, canines, and first molars of both upper and lower jaws, the bicuspid being scarcely implicated at all; the second molars are also quite healthy. In the first molars the alterations consist of deficiency of enamel on the upper surface of the crown and the presence of spines of uncovered dentine. In the case of the incisors a considerable portion of the crown of each tooth is totally devoid of enamel, and its dentine is also deficient to some extent, so that the teeth are thin, sharp-edged, and of a dirty yellowish color. The transition from the enamel-covered to the diseased part occurs suddenly in a horizontal line at some little distance from the crown of each tooth; the position of this line or step being in each tooth nearly at the same distance from the gum. The general effect when all the teeth are seen together is as if a string had been tied around them when soft and the distal part had withered."

None of these conditions, however, are in the least degree characteristic of syphilis, the special expression of which in the mouth is to be found only in the permanent upper median incisors. For the recognition and description of the peculiarities of these teeth in the subjects of inherited syphilis we are indebted, as we are for so much else of inestimable value in the study of the disease, to Mr. Hutchinson. In 1863, in a memoir on Syphilitic Diseases of the Eye and Ear, he wrote as follows¹²⁵ concerning the symptoms which, in a suspected case, would aid in determining the diagnosis: "By far the most reliable amongst the objective symptoms is the state of the permanent teeth if the patient be of age to show them. Although the temporary teeth often, indeed usually, present some peculiarities in syphilitic children of which a trained observer may avail himself, yet they show nothing which is pathognomonic, and nothing which I dare describe as worthy of general reliance.¹²⁶ The central upper incisors of the second set are the test teeth, and the surgeon not thoroughly conversant with the various and very common forms of dental malformation will avoid much risk of error if he restricts his attention to this pair. In syphilitic patients these teeth are usually short and narrow, with a broad vertical notch in their edges and their corners rounded off. Horizontal notches or furrows are often seen, but they, as a rule, have nothing to do with syphilis. If the question be put, Are teeth of the type described pathognomonic of syphilis? I answer unreservedly that when well characterized I believe they are. I have met with many cases in which the type in question was so slightly marked that it served only to suggest suspicion, and by no means to remove doubt; but I have never seen it well characterized without having reason to believe that the inference to which it pointed was well founded."

¹²⁵ Chapter on "The Means of Recognition of the Subjects of Hereditary Syphilis during the Tertiary Stage," p. 204. Before this, however, he had called attention to the same peculiarities in a paper on "The Means of Recognizing the Subjects of Inherited Syphilis in Adult Life," Medical Times and Gazette, London, Sept. 11, 1858, p. 265.

¹²⁶ So far as I know, the only recorded instances by reliable observers of the temporary teeth presenting the peculiar characteristics of syphilis are as follows: In the Transactions of the Odontological Society of Great Britain, vol. ix., 1877, p. 258, Mr. Oakley Coles described a case—without, unfortunately, giving details—in which the "peg-shaped temporary teeth were very characteristic of syphilis." Mr. Coles's abilities are well known, and the fact that at the previous meeting the society had been addressed by Mr. Hutchinson on this very subject would seem to indicate that he was quite familiar with the importance of his statement.

M. Fournier calls attention (Archives de Derm. et Syph., Sept. 25, 1883) to a cast (No. 48) to be found in the collection of M. Parrot in the museum of the Foundling Hospital at Paris. This displays the dentition of a child twenty-seven months old, in which the two upper median incisors are typical Hutchinson teeth. M. Fournier adds that later researches show unmistakably that the temporary teeth may be affected by hereditary syphilis in the same manner as those of the second dentition, although not more than one case of the former is recognized to fifteen or twenty of the latter. He thinks, however, that this proportion would be greatly modified if in the autopsies of young children the alveoli were opened to examine the embryonic teeth. M. Parrot by this means claims to have often discovered lesions of the milk teeth.

As a matter of course, so positive a statement in a matter of such gravity and importance excited considerable criticism, and the views of Mr. Hutchinson have never been without earnest and often able opponents; but it is safe to say that time has only served to place them on a surer foundation and to enhance their value in the eyes of the profession. That they have undergone no material change in the mind of their distinguished author is shown by his expressions of opinion during the debate on syphilis in the London Pathological Society in 1876,¹²⁷ and still later by the following memoranda which he gives as a guide in diagnosis:¹²⁸

"1. No special peculiarities are to be looked for in the first set of teeth.

"2. There can be no more serious blunder than to imagine that bad teeth in proportion to their badness of form are to be suspected of syphilis.

"3. The upper central incisors are the only teeth which are positively characteristic. The others may afford corroborative testimony, but are not to be relied upon alone.

"4. The chief peculiarity is a general dwarfing of the tooth, which is both too short and too narrow, and, from its sides slanting together, presents a tendency to become pointed. This tendency to pointing is always defeated by the cutting off of the end, the truncation being usually effected in a line curved upward, so as to produce a single shallow notch. At the bottom of this notch the enamel is deficient and the dentine exposed, but there is no irregular pitting, as in stomatitis teeth.

"5. The malformations are unusually symmetrical and affect pairs of teeth. The two central incisors resemble each other, and the two laterals are also alike. If any defect passes horizontally across all the incisors at the same level, and affects them all alike, it is probably not due to syphilis.

"6. In syphilis the lateral incisors usually show little or no malformation.

"7. The occurrence of the peculiarities due to syphilis and those due to mercury in the same mouth are exceedingly common."

¹²⁷ London Lancet, 1876, pp. 56 and 535.

¹²⁸ Illustrations of Clinical Surgery, fasciculus xi., London, 1878.

The great importance of the subject seems to me to justify one more quotation, as showing the opinion at a very recent date of men well qualified to judge of the correctness or inaccuracy of these statements. Mr. C. Macnamara and Dr. Thomas Barlow¹²⁹ say: "The characters of the teeth are so valuable when present that it is important to have them clearly noted—the more so that, in spite of Mr. Hutchinson's clear description, they have been much misrepresented. It may be pointed out—(1) That only the upper median permanent incisors are characteristic, and sometimes only one of them is typical, of the disease; (2) that these teeth are generally a little apart, instead of being in apposition, and are more or less dwarfed; (3) that in a typical specimen the width of the cutting edge is narrower than the width of the tooth as it emerges from the gum; (4) that a typical syphilitic tooth presents a single notch, not a serrated margin; and that occasionally, if the notch has not been actually scooped out, there is a little lunula-shaped area which may readily become a notch; (5) finally, that although such teeth, when present, are absolutely pathognomonic, the existence of normal permanent upper median incisors by no means excludes the existence of hereditary syphilis."

¹²⁹ On behalf of the Collective Investigation Committee, who have issued a circular designed to elicit information as to the effect of syphilis on the civil population of Great Britain. This circular has been sent to physicians, and contains queries as to various points relating to the symptoms of inherited syphilis; among others as to the existence in any given case of "notched, dwarfed upper median incisors," which, with or without other symptoms, would establish the diagnosis of that case. The observations above quoted are explanatory of this question (The British Medical Journal, Dec. 16, 1882).

I believe this may fairly be taken to represent the general belief at the present day among those best qualified to pronounce upon the merits of the case; and I may say that it is unqualifiedly my own opinion, arrived at after some experience and considerable investigation into the literature of the subject. It is not uncommon, however, to hear doubts expressed as to the value of this sign in the diagnosis of syphilis, and at intervals articles are written or papers read to prove that it is not of the uniform and distinctively conclusive significance that has been attributed to it.¹³⁰

¹³⁰ "I cannot say more in favor of the diagnostic values of these teeth than that, when present in typical form, they have a certain weight in favor of the existence of hereditary syphilis in the given subject" (Van Harlingen, article "Syphilis" in Internat. Encyc. of Surgery, vol. ii. p. 565). "It has been the custom from time to time since Mr. Hutchinson made his observation to question the validity of his views, both as to the fact of interstitial keratitis being due to hereditary syphilis, and as to the diagnostic values of the so-called characteristic teeth. Thus, it has been asserted, not only in England, but on the Continent, and especially in Germany, that the disease may be the result of malnutrition in scrofulous and rickety subjects; and it has been maintained that the malformation of the teeth is the simple arrest of development in a perverted constitution from other causes than syphilis" (Bumstead and Taylor, op. cit., p. 701). Garretson says (Oral Surgery, p. 316): "Observations will be found to greatly vary concerning the existence of any constancy in phenomenal expressions of the teeth in this relation."

M. Magitot, who has for some years been supposed to pay especial attention to this subject, has recently written an elaborate paper (Gazette des Hôpitaux, Sept. 29, Oct. 4, 11, and 18, 1881) to prove (1st) that dental erosions, as he calls them, are not due to syphilis; and (2d) that they are due to, or are almost invariably associated with, infantile convulsions. He has collected a number of interesting facts, but a very careful study of his article and inspection of his diagrams have convinced me that he does not recognize at all the special peculiarities of the Hutchinson teeth, but includes under his title of "erosion" a variety of widely-differing conditions. He has altogether misinterpreted Mr. Hutchinson's views as stated in his Memoirs on Certain Diseases of the Eye and Ear due to Inherited Syphilis, and represents him as at that time (1863) believing that the cause of the change in the incisors was a mercurial stomatitis. The quotation on p. 294 sufficiently refutes this absurdity. Of course the paper as an argument against the syphilitic origin of these teeth is without the slightest value.

Corroborative evidence—which, to my mind, is very strong as coming from men whose opportunities for observation of dental peculiarities are almost unlimited—is found in the writings of those gentlemen who have devoted themselves to dental and oral surgery exclusively. Mr. Henry Moon¹³¹ says:¹³² "My observations on this subject extend over some twelve years, and include some hundreds of cases; and although, in some details as to the manner of causation, I may differ from the view published by Mr. Hutchinson (before knowledge on tooth-development was advanced as it is at present), yet I must coincide entirely with his general conclusions." "The question really is this: Is there one peculiar conformation of the teeth due to inherited syphilis and not produced by any other cause? The evidence in favor of an affirmative answer to this question appears to me to be so strong that I think the onus of disproof rests with the sceptics."¹³³ These views were coincided in by the majority of the gentlemen to whom they were addressed, all of them dentists and surgeons of experience and repute, and who included men so well known to the profession as Mr. Oakley Coles, Mr. Samuel Cartwright, Mr. Charles Tomes, and others.

¹³¹ Author of the section on "Surgery of the Teeth" incorporated in Bryant's Surgery.

¹³² Proceedings of the Odontological Society of Great Britain, vol. ix., 1877, pp. 238, 239. In the same journal for 1875, vol. vii. p. 17, Langdon Down says that whenever he has discovered syphilitic teeth he has "never failed to find confirmatory evidence of the syphilitic history of the case."

¹³³ He says elsewhere (Bryant's Surgery, 3d Am. ed., p. 429) that the most characteristic change in these teeth is "the lessened breadth of the cutting edge as compared with that of the neck, the vertical groove on their anterior face being often absent, and the notch on their cutting edge not being an absolutely constant feature, and being also subject to obliteration through wear."

It may be considered as well established, then, that these peculiar teeth—stunted, abnormally narrow at the cutting edge, crescentically rounded with the convexity upward, and the surface inclined upward and forward instead of backward as in normal teeth, widely separated, but converging at their lower edges—are pathognomonic of hereditary syphilis.¹³⁴ They are often described as pegged, having been likened to a row of pegs stuck in the gums. This appearance is due to the fact that they are shortened, often projecting not more than half the normal distance from the gum, and are also widely separated; which abnormalities often affect the adjoining teeth as well, and sometimes the entire dentine. It has been asserted that other specific peculiarities are to be found associated with those of the incisors, and Mr. Moon describes as characteristic, and figures¹³⁵ small dome-shaped first molars with suppressed angles and absence of enamel from the masticatory surfaces. He believes also that when the upper incisors are typical it is exceedingly rare for the lower incisors to be altogether unaffected.

¹³⁴ Mr. Hutchinson stated in 1877 that in spite of the fact that many years previously he had challenged any one to bring forward a patient with well-marked syphilitic teeth in whose history no evidence of syphilis could be found, none had come.

¹³⁵ Proc. of Odont. Soc. of Great Brit., vol. ix. pp. 241, 242; Bryant's Surgery, Am. ed., p. 429.

The approximate cause of these peculiarities in the incisors can hardly be said to have been demonstrated. Mr. Hutchinson thought at one time¹³⁶ that they were due to a stomatitis or an alveolar periostitis, but he has since changed his mind as to that point, believing now¹³⁷ that the syphilitic tooth is the result of an arrest of development in the central or first-formed portion of the dentine. The incisors being made up of these lobes or denticles, and dwarfing of the middle one taking place, the two lateral ones fall together. This accounts at once for the small size of the tooth, its shape of an inverted truncated cone, and its crescentic edge.¹³⁸ If it were due to stomatitis, it would be more likely to be equally distributed, syphilis in its late manifestations being notably unsymmetrical; there would be no rational explanation of the involvement of one or two teeth while those on either side so frequently escaped; if it were mercurial stomatitis, the enamel too would be involved, as is not usually the case in the syphilitic teeth. It is possible that the central incisors are chiefly affected because they, with the first molars—also affected according to Mr. Moon—and the lower incisors—not infrequently involved—are the first-formed teeth.

¹³⁶ "The physiognomonical, dental, and other peculiarities by which we recognize the subject of inherited taint when advanced beyond the period of infancy are all of them the direct consequences of special inflammations from which the patient has suffered at former periods; e.g. the synechiæ and lustreless iris of iritis; the malformed teeth of periostitis of the alveolus and dental sacs; the protuberant forehead of hydrocephalus; the flattened nose of snuffles; the pale, earthy, opaque skin of cutaneous inflammation and eruption" (Aphorisms respecting Constitutional Syphilis, 1863).

¹³⁷ Proc. of Odont. Soc. of Great Britain, vol. ix., p. 248. See also ibid., pp. 241, 242, remarks of Mr. Moon; also Monthly Review of Dental Surgery, June 15, 1877.

¹³⁸ The denticle theory of formation is not necessarily opposed by the fact that there is only a single undivided pulp-cavity in these incisors. Instances of the separate formation of processes of dentinal pulp while others are being used and worn away, all of them finally to unite in a common pulp-chamber, have been observed in lower animals, as in the molar of the elephant.

The most elaborate article upon syphilitic teeth which has appeared since Mr. Hutchinson's original memoir is one by Fournier,¹³⁹ in which, after a very broad and comprehensive consideration of the subject, he arrives at the following conclusions: The hereditary influence of syphilis shows itself in the dental system in two ways, very unequal in point of diagnostic value—viz. first, by a retardation of evolution; second, by the arrest of growth and modifications of structure. The phenomena belonging to the second class may be grouped as follows: First, dental erosion. This is due to imperfect formation of the tooth, the result of a temporary stoppage in its development; but as it produces an appearance like that of worm-eaten wood, it has been called erosion, though in so far as the word conveys the idea of the wearing of a surface which has been previously normal, it is incorrect. The tooth affected with syphilitic erosion has never been normal. The different forms of erosion can be subdivided into groups according as they affect the face or the free edge or grinding surface of the tooth. Of those involving the face there are four types: Erosions en cupule, consisting of small excavations or cups in the surface of the crown; erosions en facettes, in which the surface presents a series of small planes, as though they had been filed; erosions en sillon when there is a linear excavation in the crown of the tooth in the shape of a transverse groove; and erosions en nappe, in which the whole surface is discolored, disorganized, and honeycombed.

¹³⁹ Archives de Derm. et Syph., Sept. 25, Oct. 2, Oct. 9, 1883. A translation made by the writer may be found in the Dental Cosmos for January and February, 1884.

A second group of erosions affects the free edge of the tooth, and includes the Hutchinson teeth, with several less important varieties. Dental erosions are multiple, symmetrical, maintain the same level on the crowns of corresponding teeth, and are situated at different heights on the crowns of teeth of different classes. It is evident, therefore, that they are the result of a morbid influence of a general character. There are three theories as to their etiology: (a) that they have no relation to syphilis,¹⁴⁰ but are always connected with infantile eclampsia; (b) that they are exclusively the result of hereditary syphilitic influence; and (c) that they are simply ordinary lesions originating from syphilis with marked frequency, and even in one form—the Hutchinson tooth—appearing to originate only from it. This latter view is the one adopted by Fournier himself. Continuing to group the symptoms due to arrest of growth and modification of structure, we have, second, microdontism, or dwarfing and stunting of the teeth—pegged teeth; third, dental amorphism, in which the teeth are strangely distorted or even transformed in type; fourth, dental vulnerability, or extreme susceptibility to all traumatic or disintegrating influences.

¹⁴⁰ M. Magitot, Treatise on the Anomalies of the Dental System, Paris, 1877; Clinical Studies on Erosion of the Teeth considered as a Retrospective Sign of Infantile Convulsions, Paris, 1881; Castanié, Paris, 1879, Thesis No. 384; Rattier, Paris, 1879, Thesis 569; and others.

Interstitial Keratitis.—The frequency of this form of diffuse inflammation of the cornea, and the diagnostic significance which has been so positively attributed to it—and has been as positively denied—render it of special interest to the general practitioner, who is almost certain to meet with occasional cases, and should be prepared to recognize its possible relation with other, and often graver, conditions.

It begins, commonly, as a slight, diffused haziness situated in the substance of the cornea itself, usually not far from the centre, and at first affecting only one eye. This depends at this stage on the presence of a number of little distinct dots of inflammation, limited to circumscribed, almost microscopic, areas, but later, in a few days, these coalesce, and at the end of a few weeks the whole cornea will probably have become nearly or quite opaque, looking like ground glass. There is no ulceration, and but little congestion as compared with that seen in other inflammatory diseases of the eye, although in the majority of cases there is a fulness of the ciliary vessels and a little photophobia with pains around the orbit. This condition may persist for one or two months, after which the other cornea is nearly always attacked,¹⁴¹ and is similarly affected, although the disease is apt to pass through its different stages rather faster than in the first eye.

¹⁴¹ In 91 out of 102 cases in Mr. Hutchinson's series—in 6, the left alone; in 5, the right alone (op. cit., p. 123).

When the height of the disease is reached the corneæ are nearly opaque, a bare perception of light remaining, so that the patient is just aware of the difference between its presence and absolute darkness. Then the cornea which was first involved begins to clear; this is soon followed by improvement in the other one, which in the course of a year or two results in a return to fairly good sight, although in most cases there remain a slight haziness and an abnormal expansion of the cornea.

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 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 it¹⁴² are certain forms of inflammation following small-pox and very superficial ulcers in a healing stage, together with cases of vascular conjunctiva.

¹⁴² Op. cit., p. 128.

There is also said¹⁴³ 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.

¹⁴³ Edward Nettleship in Hill and Cooper, op. cit., p. 267.

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.¹⁴⁴ Mr. Hutchinson's conclusions, drawn from an analysis of 102 cases of interstitial keratitis, bear strongly upon this point, and are as follows:¹⁴⁵ 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.

¹⁴⁴ "To those who have not had opportunities for observation at an ophthalmic hospital I would recommend that the diagnosis should be held to be doubtful if the patient does not present the peculiarities of teeth and physiognomy which I have described, since we find that the latter are the almost invariable concomitants of the true disease" (Mr. Hutchinson, op. cit., p. 128).

¹⁴⁵ Op. cit., pp. 109-129.

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.

Mr. James Dixon,¹⁴⁶ 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,¹⁴⁷ Noyes,¹⁴⁸ Förster,¹⁴⁹ Macnamara,¹⁵⁰ De Wecker,¹⁵¹ and Carter¹⁵² may be mentioned as having ranged themselves upon this side. On the other hand we have Schweiger,¹⁵³ Maunther,¹⁵⁴ Sæmisch,¹⁵⁵ Soelberg Wells,¹⁵⁶ 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.

¹⁴⁶ Article on "Diseases of the Eye," Holmes's System of Surgery, Am. ed., vol. ii. p. 71.

¹⁴⁷ Op. cit.

¹⁴⁸ Text-book of Ophthalmology.

¹⁴⁹ Handbuch der gesam. Augenheilkunde, vol. vii. p. 186, 1876.

¹⁵⁰ Op. cit.

¹⁵¹ Ocular Therapeutics, trans. of Forbes, 1879, p. 124.

¹⁵² He even asserts the converse to be true, which is going beyond what I believe can be established in regard to the invariable connection between the two diseases. He says: "The subjects of what we call simply inherited syphilis are liable—nay, are almost sure—to suffer from a peculiar form of interstitial keratitis."

¹⁵³ "Hutchinson's view, that this form of keratitis is to be regarded as the direct consequence of congenital syphilis, has not been generally accepted in Germany" (Handbook of Ophthalmology, p. 298).

¹⁵⁴ Zeissl's Jahrbuch der Syphilis, 1875, p. 288.

¹⁵⁵ Graefe and S.'s Handbuch d. Augenheilkunde, 1875, vol. iv. p. 264.

¹⁵⁶ Diseases of the Eye, p. 138.

Probably the conclusions of Jullien¹⁵⁷ 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.

¹⁵⁷ Op. cit., p. 1013.

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"¹⁵⁸—the statistics of Förster,¹⁵⁹ 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.

¹⁵⁸ Op. cit., Preface, p. x.

¹⁵⁹ Graefe and Sæmisch's Handbuch, vii., Part I.

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 the permanent teeth had not been cut, and in the remaining 61 the state of the teeth was not recorded.¹⁶⁰

¹⁶⁰ Note by Mr. Nettleship, Hill and Cooper, op. cit., pp. 263, 264.

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.

Heubner¹⁶¹ 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.

¹⁶¹ Ziemssen, vol. xii.

Althaus¹⁶² 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.

¹⁶² Medical Times and Gazette, Nov. 10, 1877.

Hutchinson makes a similar division of lesions and symptoms,¹⁶³ and the observations of Jaksch, Wilks, and Hughlings Jackson more or less closely coincide with this general classification.

¹⁶³ Ibid., Feb. 17, 1877.

Now, in spite of certain striking differences—more apparent than real, however—between inherited and acquired syphilis as regards cause, 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,¹⁶⁴ Graefe,¹⁶⁵ Jackson,¹⁶⁶ Heubner,¹⁶⁷ Dowse,¹⁶⁸ and Hutchinson,¹⁶⁹ though comparatively few in number, have already demonstrated the correctness of this supposition.

¹⁶⁴ Trans. of Path. Soc. of London, vol. xxviii., 1877.

¹⁶⁵ Archiv für Ophthalmologie, Bd. 1, Ab. i.

¹⁶⁶ Journal of Mental Science, Jan., 1875.

¹⁶⁷ Ziemssen, vol. xii.

¹⁶⁸ Syphilis of the Brain and Spinal Cord, London, 1879, chapter on "Hered. Syph.," p. 67.

¹⁶⁹ Med. Times and Gazette (? Feb. 17, 1877).

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,¹⁷⁰ 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.

¹⁷⁰ Op cit., pp. 71-75.

Barlow's two cases were both very young children, and are extremely convincing.¹⁷¹ 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.

¹⁷¹ Transactions of the Pathological Society of London, 1877, vol. xxviii. pp. 287-291.

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, 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 reports¹⁷² 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.

¹⁷² Op. cit., p. 253.

Other cases reported by the same author, numerous instances of nervous troubles in inherited syphilis reported by Hughlings Jackson,¹⁷³ Fournier,¹⁷⁴ Henoch,¹⁷⁵ and Hutchinson,¹⁷⁶ 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.

¹⁷³ Journal of Mental Science, Jan. 8, 1875; Transactions of St. Andrew's Med. Graduates' Ass., vol. i., 1868.

¹⁷⁴ Annales des Derm. et Syph.

¹⁷⁵ Nouveau Dict. de Méd. et Chirurgie, p. 885.

¹⁷⁶ Op. cit.

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.

Hill¹⁷⁷ 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.

¹⁷⁷ Op. cit., p. 254.

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.¹⁷⁸

¹⁷⁸ Ibid., p. 255.

Hughlings Jackson only found 1 case of inherited syphilis among 80 cases of chorea. The most carefully recorded cases of the latter affection associated with hereditary syphilis are two reported by Alison.¹⁷⁹

¹⁷⁹ American Journal of the Medical Sciences, July, 1877.

Syphilis of the Spleen.—Disease of this organ in inherited syphilis is especially important from two points of view. It is a valuable aid to diagnosis, and by its size and the degree of persistence of the swelling gives an approximate indication of the severity of the case.

Attention was first called to the frequency and importance of enlargement of the spleen in early hereditary syphilis by Gee in a paper read before the Royal Medical and Chirurgical Society in 1867.¹⁸⁰ He gave the histories of thirteen children in support of the statement that such enlargement occurred in almost one-fourth of all cases of hereditary syphilis, sometimes with, sometimes without, enlargement of the liver and lymphatic glands. According to him, the degree of splenic enlargement may be taken as a sort of index of the severity of the cachexia; the majority of cases with great enlargement die, but sometimes such children survive, the spleen gradually diminishing in size as the health improves—not diminishing, however, pari passu with such improvement, but remaining for a long time "a monument of past cachexia."

¹⁸⁰ British Medical Journal, 1867, vol. i. p. 435.

Barlow,¹⁸¹ ten years later, thought Gee had rather understated the proportion of cases in which splenic enlargement occurs, he having found it in 22 out of 28 children with definite hereditary syphilis. Birch-Hirschfeld, Eisenschitz, and Tepel¹⁸² corroborate these observations, finding that the enlargement is almost invariable and that the spleen is often double its normal size.

¹⁸¹ Trans. of Path. Soc. of London, Jan. 20, 1877.

¹⁸² Quoted by Hill and Cooper, op. cit., pp. 164, 165.

Mr. W. J. Tyson has reported¹⁸³ a cure of a child born of syphilitic parents, in whom, at two years of age, the spleen extended downward three and a half inches, reaching the crest of the ilium and approaching closely to the umbilicus. The liver was not enlarged; the urine was not albuminous. He ordered mercury with chalk, one grain every morning and evening, and one grain of iodide of potassium, with ten minims of syrup of iodide of iron to an ounce of water, three times a day. Sixteen months later the spleen had become imperceptible, and three years afterward the child was in excellent health.

¹⁸³ The Lancet, Oct. 23, 1880.

The diminution of the liver under treatment appears to take place before there is any diminution in the size of the spleen.¹⁸⁴ This persistence of the latter renders it, as has been stated, a valuable diagnostic sign. In the paper already quoted from,¹⁸⁵ Macnamara and Barlow allude to this as follows: Enlargement of the liver, although it ought to be noted because it is often present in hereditary syphilis, has but little value as a confirmatory symptom—first, because the liver is proportionally large in infancy, and it is difficult to state the limit of what is actually normal; and, secondly, because other causes besides congenital syphilis lead to its enlargement.

¹⁸⁴ Barlow, British Medical Journal, Jan. 20, 1877.

¹⁸⁵ British Medical Journal, Dec. 16, 1882.

With regard to enlargement of the spleen the case is different. Gee's observation, that in the early stage of infantile syphilis some enlargement of the spleen occurs in a large number of cases, has been abundantly confirmed. Although with the subsidence of the other symptoms this enlargement often disappears, so that on post-mortem examination two or three months after there may be no trace of it, yet in a few cases it persists, and indeed sometimes increases, so as to be considerable when the other signs have quite vanished. The importance of this sign is greatest when noted early; as, for example, when the child is from two to three months old, for at that period the enlargement of the spleen due to rickets can hardly come into question.

The condition of the spleen during this period of enlargement seems to be simply that of hyperæmia, or at the most of hyperplasia, Gee's, Barlow's, and Birch-Hirschfeld's autopsies showing no evidence of new growth or of amyloid or other changes.¹⁸⁶

¹⁸⁶ Parrot reports (Le Mouvement méd., Paris, Nov. 23, 1872) two forms of splenic disease produced by inherited syphilis: 1. A simple hypertrophy, which he thinks is secondary to diffuse infiltration of the liver, obstructing the portal circulation and causing the spleen to act as a reservoir; 2. An inflammatory condition resulting in the formation of false membranes around the capsule. His explanation of the first condition is unsatisfactory, because there are numerous cases in which the spleen is enlarged without any involvement of the liver. His other observations have never been confirmed.

The cause seems to me to be in all probability the well-known effect of syphilis on the glandular system in general, and the lymphatic system in particular, to which I believe the spleen is now generally assigned. The analogy between this slow, persistent, painless enlargement preceding the cutaneous symptoms,¹⁸⁷ unaccompanied by inflammatory symptoms, unattended by any breaking down of tissue, subsiding slowly but evenly under specific treatment, and the behavior of the buboes of acquired syphilis, is certainly very striking.¹⁸⁸

¹⁸⁷ Eisenschitz, Wiener med. Wochenschrift, Nos. 48 and 49.

¹⁸⁸ A similar enlargement occurs, but much more rarely, in the secondary period of acquired syphilis. Weaver noted it in 3 out of 79 soldiers suffering from early syphilis. Wilks and Moxon report cases in which the average weight was 19 oz.

In most cases of hereditary syphilis there are evidences of disturbance of the gastro-intestinal tract. Vomiting, diarrhoea, colic, anorexia, and emaciation are well-known, but of course not at all characteristic, symptoms.

It has been supposed that the mucous membrane of the entire tract was probably, during the early period at least, and coincidently with the cutaneous eruption, in a condition of hyperæmia and irritation comparable to that of the skin.¹⁸⁹ Whether this be so, or whether it is due to associated involvement of the glandular apparatus, has not yet been determined.

¹⁸⁹ "There is surely no a priori probability that a blood disease so severe as syphilis should produce lesions on the skin, in the mouth, and in the eye only—that it should, in fact, affect all the visible parts and avoid all the concealed ones" (Mr. Hutchinson, The Lancet, Feb. 6, 1876).

Förster¹⁹⁰ has found fibroid degeneration of Peyer's patches in a syphilitic infant who died six days after birth, the glandular structure having been replaced by elevated grayish-red masses of nuclei, cells, and connective-tissue fibre. Ulcers of the intestines have been described, but appear to have had no specific characters.

¹⁹⁰ Quoted by Bumstead and Taylor, op. cit., p. 757. His observations were confirmed by Eberth, Roth, and Oser.

The pancreas has been most extensively studied by Birch-Hirschfeld, who examined seventy-three syphilitic foetuses. In thirteen of them he found enlargement of the pancreas with increased weight and density, proliferation of connective tissue, and in some cases compression—almost obliteration—of the lobules, with atrophy and fatty degeneration of their epithelium.¹⁹¹

¹⁹¹ Klebs discovered a gumma in the pancreas of a six-months' foetus.

The peritoneum has been described by Simpson, Von Baerensprung, and others as occasionally invaded in early hereditary syphilis. There seems to be no evidence that it is ever directly affected, the cases in which death occurs from peritonitis being due usually to trouble connected with the liver or spleen.

Syphilis of the lung, originally described by Depaul and Virchow, has been carefully studied also by Förster, Robin, Lorain, and Cornil, from whose description¹⁹² of the pathology of the condition I condense the following: In the syphilitic foetus born before term, in the syphilitic child born dead at full term, and in the syphilitic children who live a few days, there are found at the autopsy, in the lungs, nodules or tumors, usually superficial, sometimes deep, hard, isolated or in groups, pink, gray, or red in color, with scattered whitish or yellowish points. Their size varies from a pea to a small walnut. They represent a portion of the lung more or less considerable in a state of special lobular hepatization. Sometimes a whole lobe is involved. The affected portion is very dense and covered with thickened inflamed pleura. The lesions are those of chronic pneumonia. The interlobular connective tissue enters into proliferation and presents a large quantity of embryonal cells; the alveolar walls are thick, while the narrow alveoli are livid, and even filled by epithelial cells, which are of the pavement form in contact with the walls, round in the centre of the alveoli. As the process progresses the epithelial cells become fatty, degenerated, and subsequently broken down and absorbed, while the embryonal interalveolar tissue rapidly organizes into fibrous tissue. Thus results a small fibrous tumor, in which a gumma may ultimately develop.¹⁹³

¹⁹² Syphilis, trans. of Simes and White, Philada., 1882, p. 404.

¹⁹³ Cornil and Ranvier's Path. Histology, Am. ed., 1880.

It is impossible to confound this syphilitic pneumonia with tuberculosis. The granulations of tubercle are never congenital.