the observations of Jaksch, Wilks, and Hughlings Jackson more or less
closely coincide with this general classification.
[Footnote 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,
{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,[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.
[Footnote 164: _Trans. of Path. Soc. of London_, vol. xxviii., 1877.]
[Footnote 165: _Archiv für Ophthalmologie_, Bd. 1, Ab. i.]
[Footnote 166: _Journal of Mental Science_, Jan., 1875.]
[Footnote 167: _Ziemssen_, vol. xii.]
[Footnote 168: _Syphilis of the Brain and Spinal Cord_, London, 1879,
chapter on "Hered. Syph.," p. 67.]
[Footnote 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.
[Footnote 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.
[Footnote 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, {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[172] 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.
[Footnote 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.
[Footnote 173: _Journal of Mental Science_, Jan. 8, 1875; _Transactions
of St. Andrew's Med. Graduates' Ass._, vol. i., 1868.]
[Footnote 174: _Annales des Derm. et Syph._]
[Footnote 175: _Nouveau Dict. de Méd. et Chirurgie_, p. 885.]
[Footnote 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.
Hill[177] 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.
[Footnote 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]
[Footnote 178: _Ibid._, p. 255.]
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.[179]
[Footnote 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."
[Footnote 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 Tepel[182] corroborate these
observations, finding that the enlargement is almost invariable and
that the spleen is often double its normal size.
[Footnote 181: _Trans. of Path. Soc. of London_, Jan. 20, 1877.]
[Footnote 182: Quoted by Hill and Cooper, _op. cit._, pp. 164, 165.]
Mr. W. J. Tyson has reported[183] 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.
[Footnote 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.
[Footnote 184: Barlow, _British Medical Journal_, Jan. 20, 1877.]
[Footnote 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 {306} 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]
[Footnote 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]
[Footnote 187: Eisenschitz, _Wiener med. Wochenschrift_, Nos. 48 and
49.]
[Footnote 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.
[Footnote 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).]
Förster[190] 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.
[Footnote 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
{307} 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]
[Footnote 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 description[192] 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]
[Footnote 192: _Syphilis_, trans. of Simes and White, Philada., 1882,
p. 404.]
[Footnote 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.
The fact that in syphilitic interstitial hyperplasia the change begins
in the interlobular connective tissue and around the interlobular
vessels, at first consisting of small spindle-shaped and roundish cells
which quickly develop into connective tissue, and the fact that
blood-vessels are freely produced among the fibres of this new tissue,
seemed, in the estimation of those pathologists who took part in the
discussion on visceral syphilis in 1877, to constitute its most
distinctive feature.[194]
[Footnote 194: _Trans. of Lond. Path. Soc._, vol. xxviii.--views of
Green, Jones, Greenfield, Moxon, and others.]
Gummata in the lungs of children suffering from inherited syphilis have
been described by a number of writers. They appear likewise to begin in
the walls of the blood-vessels or the bronchioles. They differ from
tubercular nodules in being few in number--not more than half a dozen
usually--and are generally confined to one lung.
The condition of the arteries in the few cases in which they have been
noticed as affected by inherited syphilis was precisely similar to that
found by Heubner in the arteries of adults.[195]
[Footnote 195: See Cornil, _op. cit._, p. 305.]
{308} The symptoms are very variable; new-born children often die
asphyxiated in a few days. If they live longer, the disease develops
into a more generalized broncho-pneumonia.
Syphilis of the Larynx.--The hoarse cry of the new-born infant so
characteristic of hereditary syphilis depends upon the presence of
hyperæmia, of mucous patches, or even of extensive ulceration. I am
inclined to think that the first is the more common, as if it were
otherwise cases of death from oedema glottidis or other forms of
laryngeal obstruction would be oftener met with. When ulceration does
exist it is generally, but not invariably, secondary to pharyngeal
ulcers.[196]
[Footnote 196: See synopsis of six cases of George M. Lefferts,
reported in Bumstead and Taylor, _op. cit._, p. 754.]
Bronchial catarrh, giving rise to cough, and sometimes to considerable
embarrassment of respiration, is a not infrequent complication of
laryngeal syphilis.[197]
[Footnote 197: Schnitzler, _Die Lungen Syphilis, etc._, 1880, S. 41.]
Later troubles of the larynx in connection with inherited syphilis have
not yet been carefully enough studied to warrant us in drawing any
distinction between them and the usual symptoms seen in the acquired
disease.
Syphilis of the testicles has been studied by Henoch,[198] Cornil,[199]
Parrot,[200] Hutinel,[201] North,[202] Bryant,[203] and others. It is
found to consist of a true interstitial orchitis, very closely
resembling that seen in the syphilitic testicles of adults. Hutinel's
investigations, based on ten cases, showed the testicles slightly
enlarged and harder than normal, the scrotum pendent, the epididymis
normal, the tension of the tunica vaginalis and tunica albuginea
slight. The basis of the lesion is in a collection of small round
embryonal cells resembling lymph-cells, arranged in the connective
tissue around the arterioles which come from the tunica albuginea. This
may be accompanied by a more or less marked diffused interstitial
orchitis, or there may be only a thickening from the new formation of
small round cells on the connective tissues of the testicles. Cornil
found the seminal ducts separated by numerous round or fusiform cells.
The disease usually occurs at from two months to three years of age;
both testicles are generally involved, and are enlarged, hard,
inelastic, and frequently nodulated. Mercurial treatment generally
causes a marked improvement unless the inflammation has already
resulted in the development of a new fibroid formation, in which case
it would be likely to remain unaffected by any form of treatment.
Inunctions with diluted mercurial ointment, iodoform, etc. are useful
adjuvants.
[Footnote 198: _Schmidt's Jahrbuch_, 178, No. 4.]
[Footnote 199: _Op. cit._, p. 420.]
[Footnote 200: _Rev. mens. de Méd. et de Chir._, Paris, Feb., 1878.]
[Footnote 201: _Ibid._]
[Footnote 202: _Med. Times and Gaz._, Lond., 1862, vol. i. p. 403.]
[Footnote 203: _Ibid._, Dec., 1863.]
The kidneys are not infrequently involved in inherited syphilis. Parrot
reports the pathological change to consist of a proliferation of small
round cells in the intertubular connective tissue, followed by
contraction, obliteration of the tubules, and degeneration of their
epithelium.
Bradley has reported[204] the case of a child aged four months in whom
a well-marked syphilitic eruption and an attack of acute Bright's
disease were coincident. Mercurial treatment for three weeks cured
both.
[Footnote 204: _British Med. Journ._, Feb. 4, 1876.]
Coupland has reported two cases of parenchymatous nephritis associated
with inherited syphilis, but advances no proof that it was not an
accident. {309} Gummata have been from time to time found in the
kidneys of very young children who have died from their effects and
from other visceral lesions due to syphilis.[205] Cases of enlargement,
of fibroid, fatty, and gelatinous degenerations of the suprarenal
capsules, have been recorded.
[Footnote 205: See discussion in Clinical Soc. of London, Jan., 1880;
"Remarks on Visceral, and especially on Renal, Syphilis," by
Barthelémy, _Annales de Derm. et Syph._, April, 1881.]
The thymus gland is occasionally found in syphilis to have undergone
alterations claimed by Dubois, Depaul, and others to be syphilitic in
their nature, but ascribed by Parrot simply to degenerative changes due
to malnutrition. The gland does not appear to undergo any marked
alteration in size, color, or consistency, but is found after death to
contain a small quantity of purulent matter.
The tendency of syphilis is certainly not, as a rule, to the formation
of pus. Nearly all the lesions we have studied with the exception of
breaking-down gummata have consisted in various forms of
cell-proliferation or accumulation, and not in the formation of
abscesses, and it is not probable that this is an exception. I doubt
very much the syphilitic character of these changes.[206]