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 Moon[131] 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.
[Footnote 131: Author of the section on "Surgery of the Teeth"
incorporated in Bryant's _Surgery_.]
[Footnote 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."]
[Footnote 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 {297} 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 figures[135] 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.
[Footnote 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.]
[Footnote 135: _Proc. of Odont. Soc. of Great Brit._, vol. ix. pp. 241,
242; Bryant's _Surgery_, Am. ed., p. 429.]
[Illustration: FIG. 11. Serrations of Normal Incisors.]
[Illustration: FIG. 12. Syphilitic Incisors.]
A mistake which I know, from observation, to be frequently made is the
confusing of the normal serrations of the cutting edges of
recently-erupted normal incisors with the peculiar crescentic edges of
the syphilitic teeth. It seems worth while to call especial attention
to this, on account of the unpleasant consequences which often follow
injudicious questioning based on such supposed syphilitic phenomena.
Indeed, the space which has been devoted to this subject of syphilis of
the teeth is well warranted, I am sure, by the fact that not only do
diagnosis, prognosis, and treatment in cases of great severity, and in
both children and parents, often depend upon a recognition of these
peculiarities, but that in addition to the clinical and therapeutic
problems there are others the solution of which is dependent upon the
same knowledge on the part of the practitioner, and which may involve
reputation, marital relations, and personal honor and happiness.
The approximate cause of these peculiarities in the incisors can hardly
be said to have been demonstrated. Mr. Hutchinson thought at one
time[136] that they were due to a stomatitis or an alveolar
periostitis, but he has since changed his mind as to that point,
believing now[137] 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 {298} 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.
[Footnote 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).]
[Footnote 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.]
[Footnote 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.
[Footnote 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
{299} which the teeth are strangely distorted or even transformed in
type; fourth, _dental vulnerability_, or extreme susceptibility to all
traumatic or disintegrating influences.
[Footnote 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.
[Footnote 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 {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[142] are certain forms of inflammation following
small-pox and very superficial ulcers in a healing stage, together with
cases of vascular conjunctiva.
[Footnote 142: _Op. cit._, p. 128.]
There is also said[143] 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.
[Footnote 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.
[Footnote 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).]
[Footnote 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.
{301} 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 Carter[152] 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.
[Footnote 146: Article on "Diseases of the Eye," Holmes's _System of
Surgery_, Am. ed., vol. ii. p. 71.]
[Footnote 147: _Op. cit._]
[Footnote 148: _Text-book of Ophthalmology_.]
[Footnote 149: _Handbuch der gesam. Augenheilkunde_, vol. vii. p. 186,
1876.]
[Footnote 150: _Op. cit._]
[Footnote 151: _Ocular Therapeutics_, trans. of Forbes, 1879, p. 124.]
[Footnote 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."]
[Footnote 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).]
[Footnote 154: Zeissl's _Jahrbuch der Syphilis_, 1875, p. 288.]
[Footnote 155: Graefe and S.'s _Handbuch d. Augenheilkunde_, 1875, vol.
iv. p. 264.]
[Footnote 156: _Diseases of the Eye_, p. 138.]
Probably the conclusions of Jullien[157] 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.
[Footnote 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.
[Footnote 158: _Op. cit._, Preface, p. x.]
[Footnote 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 {302} the permanent teeth had not been cut, and in the remaining
61 the state of the teeth was not recorded.[160]
[Footnote 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.
Heubner[161] 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.
[Footnote 161: _Ziemssen_, vol. xii.]
Althaus[162] 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.
[Footnote 162: _Medical Times and Gazette_, Nov. 10, 1877.]