then associated with dwarfishness and a defective development of the
cranium. Generally, however, it is an extra-uterine affection,
originating mostly during infancy and childhood, though it is also met
with at the age of puberty, and even in adult life. The male sex is
more predisposed to the affection than the female. When hypertrophy of
the brain occurs during infancy, it is almost always associated with
an excessive development of the lymphatic glands, with a defective
involution of the thymus gland, and with rachitis, but generally
without cachexia. No special exciting cause can be assigned to the
affection during youth or adult age, though it has been stated that
during these periods of life the disease may supervene upon tubercle
or carcinoma of the brain. Repeated active or passive congestion
(Rokitanski, Andral), as well as the introduction of lead into the
system, has also been supposed to incite the disease.
PATHOLOGICAL ANATOMY.—Directly after the removal of the vault of the
cranium, and on cutting through the dura mater, the brain, as if
liberated from pressure, is observed to swell out to such a degree as
to render the replacement of the removed skull-cap impossible. The
enveloping membranes are found closely adapted to one another and to
the brain; they are very thin, bloodless, and dry, and their vessels
are empty and pressed flat. The hemispheres of the cerebrum are large,
and their convolutions, mutually pressing against one another, are
flattened at their surfaces, so that the intervening sulci are hardly
recognizable. After the removal of the brain from the skull the
abnormal dimensions of the cerebrum present a remarkable contrast to
the normal size of the cerebellum, pons, and medulla oblongata, which
also appear flat and broad from pressure. When a horizontal section is
made through the hemispheres of the cerebrum and a little above the
corpus callosum, the centrum ovale appears unusually large. The
cavities of the ventricles are very narrow, their walls touching one
another. There is no serum in the pia mater or in the ventricles. The
substance of the brain is pale, bloodless, and dry. The white
substance, upon which the hypertrophy particularly depends, is of a
doughy consistence, comparable to the boiled white of an egg or
cheese, whilst the gray substance is so pale as to be hardly
distinguishable from the former.
The condition of the skull is, according to Rokitanski,[1] as follows:
In cases in which the hypertrophy of the brain has advanced to a high
degree, and in which the sutures of the skull are united, the bones of
the cranium are found thinner and their inner table roughened by
absorption. This is especially the case in the bones which form the
vault of the cranium. The holes or other deficiencies found at the
base of the cranium in the plates of the frontal, ethmoid, and
sphenoid bones are not entirely due to the absorbing process, but
rather to the thinness of these bones. In infants the cavity of the
cranium enlarges in proportion to the hypertrophy of its contents; the
head then resembles in form so much that of hydrocephalus that it may
lead to errors in diagnosis. In some cases in which the disease
rapidly develops to a high degree there is observed on the infant's
skull a loosening and separation of the sutures of the cranial vault,
with red coloration and suffusion of their cartilages.
[Footnote 1: _Lehrbuch der Pathologischen Anatomie_, 3d ed., 1855,
vol. ii. p. 431.]
{998} SYMPTOMS.—There is a gradually increasing muscular weakness,
manifesting itself especially in the lower extremities, and giving
rise to an unsteady, stumbling gait and frequent falling, caused
perhaps by the excessive weight of the head, and also an inability of
securely grasping objects. Besides these disturbances of motion,
epileptic spasms appear, at first light in degree and at long
intervals, but becoming later on in the course of the disease more
frequent and severe. Continuous or intermittent attacks of headache
almost always accompany the disease, and, furthermore, vertigo,
tinnitus aurium, photophobia, and dimness of sight with dilatation of
the pupil; general sensation also becomes blunted, but without ever
amounting to anæsthesia. In a number of cases there is no disturbance
of the psychical functions, though in others symptoms of mental
excitement amounting even to delirium have been observed. Most
frequently the intelligence sinks from the beginning of the disease,
to end in complete idiocy. The pulse also has been observed to fall
considerably during the last stage.
The COURSE of the disease is, according to Andral and Hasse,[2] almost
always chronic, and if an acute development of the affection has been
spoken of, it may be supposed that the latter has commenced a
considerable time previous to the manifestation of the symptoms during
the last stage. Andral divides the disease into two stages, of which
the first is chronic and frequently latent, whilst the other is more
acute and leads to a rapid fatal termination. In most cases death is
caused by such intercurrent affections as give rise to irritation and
hyperæmia of the brain, and thus increase the already abnormal
pressure upon this organ.
[Footnote 2: “Krankheiten des Nervensystems,” 2d ed., 1869, in
_Handbuch der Speciellen Pathologie und Therapie_, edited by R.
Virchow, vol. iv. 1st div., p. 578.]
DIAGNOSIS, PROGNOSIS, AND TREATMENT.—It has already been mentioned
that errors in diagnosis may very easily be committed on account of
the great resemblance in the form of the head in cases of hypertrophy
of the brain and of hydrocephalus, though it has been stated that in
the former affection convulsions, in the form of epileptic spasms,
predominate, to be followed during the last stage by symptoms of
depression; whilst in hydrocephalus the symptoms of depression
manifest themselves from the beginning of the disease, and, moreover,
the rachitic deformities are more prominently shown in the form of a
chicken-breast. A positive diagnosis can only be made by the autopsy.
There are no remarks to be made on the prognosis and treatment of
hypertrophy of the brain.
{999}
SYPHILITIC AFFECTIONS OF THE NERVE-CENTRES.
BY H. C. WOOD, M.D., LL.D.
Introduction.
Syphilitic affections of the nerve-centres are best studied by
separating those of the spinal cord from those of the cerebrum, and in
the present article this natural division of the subject is adopted.
Further, cerebral syphilis in its most characteristic or gummatous
form usually attacks the brain-membranes, or perhaps in some cases the
perivascular sheaths of the vessels, and only secondarily affects the
tissue of the brain itself. The question of the occurrence of specific
disease of the brain-cortex is so important that it shall have a
separate discussion. It is perfectly well proven that with or without
other brain lesion the vessels of the brain may undergo an
atheromatous degeneration as the direct result of a syphilitic
dyscrasia; but such disease links itself on the one hand with the
subject of syphilitic disease of the general vascular system, and on
the other hand with cerebral apoplexies, softenings, and other
degenerations. Moreover, the space here allotted to brain syphilis is
very insufficient. I, therefore, shall not enter upon the further
discussion of syphilitic degeneration of the brain-vessels. The
etiology of brain and spinal syphilis is best discussed under one
heading.
* * * * *
GENERAL ETIOLOGY.—We do not know why in any individual case syphilis
selects one portion of the nervous centres rather than another for
attack; indeed, it is only rarely that any exciting cause can be
discovered.
It is not unnatural to expect that any agency which is capable of
exciting an inflammation of a nerve-centre may, when present in a
syphilitic person, provoke a specific disease of such centre. Thus,
thermic fever is a very common cause of chronic meningitis, and in the
_Journ. de Méd. et Chir._ (Paris, 1879, p. 191) a case is reported in
which cerebral syphilis followed a sunstroke; I have myself seen one
similar instance, and in Roberts's case of precocious cerebral
syphilis (see p. 804) the first convulsion came whilst the man was
fishing on a very hot day, and may have been precipitated by the
exposure.
Blows and other traumatisms would be expected to figure largely as
exciting causes of nervous syphilis, but they, in fact, are only
rarely present. I have seen one or two cases of specific brain disease
attributed to violence by the patient, and several cases of possibly
specific spinal {1000} disease—one in which a poliomyelitis followed a
fall on the ice; one in which, after a fall from a cart and marked
spinal concussion, a local myelitis developed;[1] and one of a general
myelitis following an injury by a horse. The only records of such
cases are those of Broadbent[2] and those collected by Heubner.[3]
[Footnote 1: _Univers. Hosp. Dispen. Service-Book_, x., 1875, p. 58.]
[Footnote 2: _Lond. Lancet_, 1876, ii. p. 741.]
[Footnote 3: _Ziemssen's Encyclopædia_, xii. 301.]
Various authorities attach much influence to over-study and other
forms of cerebral strain in exciting brain syphilis. Engelstedt is
stated to have reported cases having such etiological relations, and
Fournier[4] affirms that he has especially seen the disease in
professional men and other persons habitually exercising their brains
to excess. Neither in private nor public practice have I met with an
instance where over-brainwork could be considered a distinct
etiological factor, whilst I have seen some hundreds of cases from
amongst the laboring classes, in whom the intellectual faculties are
chiefly dormant.
[Footnote 4: _La Syphilis du Cerveau_.]
The drift of the evidence in medical literature is so pronounced, and
so in accord with my own experience, that I believe it may be
positively affirmed that in the vast majority of cases of nervous
syphilis no exciting cause can be found.
Inherited syphilis seems to be less prone than the acquired diathesis
to attack the nervous system, but is certainly capable of so acting.
As early as 1779, Joseph Glenck[5] reported a case of a girl, six
years old, cured by a mercurial course of an epilepsy of three years'
standing and of other manifestations of hereditary syphilis. Graefe
found gummatous tumors in the cerebrum of a child nearly two years
old.[6] O. Huebner[7] details the occurrence of pachymeningitis
hæmorrhagica in a syphilitic infant under a year old. Hans Chiari[8]
reports a case in which very pronounced syphilitic degeneration of the
brain-vessels was found in a child fourteen months old. Both Barlow[9]
and T. S. Dowse[10] report cases of nerve syphilis in male infants of
fifteen months. For other similar cases the reader is referred to an
article by J. Parrott,[11] and to a paper by M. E. Troisier.[12]
[Footnote 5: _Doctrina de Morbis Venereis_, Vienna.]
[Footnote 6: _Arch. f. Ophthalm._, Bd. i. Erst Abth.]
[Footnote 7: _Virchow's Archiv_, Bd. lxxxiv. 269.]
[Footnote 8: _Wien. Med. Wochenschrift_, xxxi. 1881, 17.]
[Footnote 9: _Lond. Patholog. Soc. Trans._, 1877.]
[Footnote 10: _The Brain and its Diseases_, vol. i. p. 76.]
[Footnote 11: _Archiv. de Physiologie_, 1871-72, p. 319; also to his
“Leçons sur le Syphilis hered.,” _Progrès méd._, 1877 and 1878.]
[Footnote 12: _Arch. de Tocologie_, x. 411.]
Recorded cases prove decisively that even after puberty specific
nervous affections may primarily attack the unfortunate offspring.
Thus, Nettleship reports[13] the development of a cerebral gumma in a
girl of ten years, and J. A.. Ormerod[14] of a tumor of the median
nerve (probably gummatous) in a woman of twenty-three, both the
subjects of inherited syphilis. Thomas S. Dowse[15] details a case of
cerebral gumma at the age of ten years, and Samuel Wilks[16] one of
epilepsy, from inherited taint, in a boy of fourteen. J.
Hughlings-Jackson reports[17] paraplegia with epilepsy in a boy of
eight, hemiplegia in a girl of eighteen, and hemiplegia in a woman of
twenty-two;[18] the nervous affection in each case being associated
with or dependent upon inherited syphilis. E. Mendel reports[19] a
case of a child {1001} who had inherited syphilis, and developed in
her fifteenth year a maniacal attack with hallucinations. I have seen
cerebral syphilis occur at twenty-one years of age as the first
evident outbreak of the inherited disorder.
[Footnote 13: _Trans. Lond. Path. Soc._, xxxii. 13.]
[Footnote 14: _Ibid._, p. 14.]
[Footnote 15: _Loc. cit._, p. 71.]
[Footnote 16: _Lectures on Dis. of Nerv. Syst._, Philada., 1878, p.
333.]
[Footnote 17: _Journ. Ment. and Nerv. Diseases_, 1875, p. 516.]
[Footnote 18: _Brit. Med. Journal_, May 18, 1872.]
[Footnote 19: _Archiv f. Psychiatrie_, Bd. i. 313.]
When a nervous affection develops first at a comparatively late
period, and no very apparent evidences of the inherited taint are
present, there is great danger of the case being misunderstood;
indeed, in some instances an immediate diagnosis may be scarcely
possible. It is probable that in most of the reported recoveries from
alleged tubercular meningitis the disease has been syphilitic.
Some time since I saw, in an orphan of fourteen, a chronic basal
meningitis, and in the absence of any history and of any evidences of
syphilis gave the fatal prognosis of tubercular disease; but, to my
astonishment, under the long-continued and free use of iodide of
potassium complete recovery occurred. Another child, reported by a
very good practitioner as cured of tubercular meningitis, and
afterward for a long time under my own care, I believe suffered from
hereditary syphilis. Cases of this character have also been reported
by F. Dreyfous.[20]
[Footnote 20: _Revue mensuelle des Malad. des Enfants_, 1883, i. 497;
see also _Gaz. hébdom. Sci. méd. de Montpellier_, 1883, v. 89.]
It is of course very important to diagnose between a tubercular
meningitis and one due to hereditary syphilis. Without a history
certainty is not possible, but a general indefiniteness of symptoms
and slowness of progression should arouse suspicion, especially if the
absence of the pulse-retardation indicated that the vault rather than
the base of the cranium was involved.
The relation of inherited syphilis to various nervous affections not
distinctly specific cannot yet be determined. Arrested development,
and the consequent epilepsy, idiocy,[21] early brain sclerosis, are
probably sometimes due to the inheritance; and the cases collected by
E. Mendel[22] show that chronic hydrocephalus is frequently of
specific origin.[23]
[Footnote 21: See _Brain_, vol. vii. 409.]
[Footnote 22: _Archiv f. Psychiatrie_, Bd. i. 309.]
[Footnote 23: See, also, _Virchow's Archiv_, Bd. xxxviii. p. 129.]
Another very important question connected with the etiology of these
disorders is as to the time of their development. Nervous diseases
following acquired syphilitic infection certainly belong to the
advanced stages of the disorder. Huebner reports[24] a case in which
thirty years elapsed between the contraction of the chancre and the
nervous explosion. I have seen a similar period of thirty years.
Fournier reports intervals of twenty-five years, and thinks from the
third to the tenth year is the period of maximum frequency of nervous
accidents.
[Footnote 24: _Ziemssen's Encyclopædia_, xii. 298, New York ed.]
The fact that nervous syphilis may occur many years after the
cessation of all apparent evidences of the diathesis is of great
practical importance, especially as the nervous system is more prone
to be attacked when the secondaries have been very light than when the
earlier manifestations have been severe. I have repeatedly seen
nervous syphilis in persons whose secondaries have been so slight as
to have been entirely overlooked or forgotten, and who honestly
asserted that they never had had syphilis, although they acknowledged
to gonorrhœa or to repeated exposure, and confessed that their
asserted exemption was due to good fortune rather than to chastity.
{1002} The following citations prove that this experience is not
peculiar. Dowse[25] says: “Often have I had patients totally ignorant
of having at any time acquired or experienced the signs or symptoms of
syphilis in its primary and secondary stages, yet the sequelæ have
been made manifest in many ways, particularly in many of the obscure
diseases of the nervous system.” Buzzard[26] reports a case of nervous
syphilis where the patient was unconscious of the previous existence
of a chancre or of any secondaries. Rinecker also calls attention[27]
to the frequency of nervous syphilis in persons who afford no distinct
history of secondary symptoms.
[Footnote 25: _The Brain and its Diseases_, London, 1879, vol. i. p.
7.]
[Footnote 26: _Syphilitic Nervous Affections_, London, 1874, p. 80.]
[Footnote 27: _Archiv f. Psychiatrie_, vii. p. 241.]
Although syphilis is prone to attack the nervous system many years
after infection, it would be a fatal mistake to suppose that nervous
disease may not rapidly follow the chancre. What is the minimum
possible intermediate period we do not know, but it is certainly very
brief, as is shown by the following cases of this so-called precocious
nervous syphilis. Alfrik Ljunggrén of Stockholm reports[28] the case
of H. R——, who had a rapidly-healed chancre in March, followed in May
of the same year by a severe headache, mental confusion, and
giddiness. Early in July H. R—— had an epileptic attack, but was
finally cured by active antisyphilitic treatment. Although the history
is not explicit, the nervous symptoms appear to have preceded the
development of distinct secondaries other than rheumatic pains.
[Footnote 28: _Archiv f. Dermatol. u. Syphilis_, 1870, ii. p. 155.]
Davaine is said[29] to have seen paralysis of the portio dura “a month
after the first symptoms of constitutional syphilis.” E. Leyden[30]
found advanced specific degeneration of the cerebral arteries in a man
who had contracted syphilis one year previously. R. W. Taylor details
a case in which epilepsy occurred five months after the infection.[31]
In the case of M. X——, reported by Ad. Schwarz,[32] headache came on
the fortieth day after the appearance of the primary sore, and a
hemiplegia upon the forty-sixth day. S. L——[33] had a paralytic stroke
without prodromes six months after the chancre. A. P. L——[34] had an
apoplectic attack seven months after the chancre; A. S——, one five
months after her chancre. In a case which recently occurred in the
practice of A. Sydney Roberts of this city the chancre appeared after
a period of incubation of twenty-six days, and two months and eight
days subsequent to this came the first fit; eight days after the first
the second convulsion occurred, with a distinct aura, which preceded
by some minutes the unconsciousness. An interesting observation in
this connection is that of Ern. Gaucher[35] of a spinal syphilis
occurring six months after the appearance of a chancre.
[Footnote 29: Buzzard, _Syphilitic Nervous Affections_, London, 1874.]
[Footnote 30: _Zeitschrift f. klin. Med._, Bd. v. 165.]
[Footnote 31: _Journ. Nervous and Mental Dis._, 1876, p. 38.]
[Footnote 32: _De l'Hémiplegia syphilitique Prêcoce_, Inaug. Diss.,
Paris, 1880.]
[Footnote 33: _Ibid._]
[Footnote 34: _Ibid._]
[Footnote 35: _Revue de Méd._, 1882, ii. 678.]
This citation of cases might be much extended, but is sufficient to
show that nervous syphilis occurs not very rarely within six months
after infection, and may be present in two months.
{1003} Gummatous Brain Syphilis.
CLINICAL HISTORY.—Brain syphilis of the type now under consideration
may declare itself with great suddenness. An apoplectic attack, a
convulsive paroxysm, a violent mania, or a paralytic stroke may be the
first detected evidence of the disease. In most of these cases the
coming storm ought to have been foreseen, and to a greater or less
degree averted. The onset of cerebral syphilis is, however, generally
more gradual, the symptoms coming on slowly and successively. Proper
treatment, instituted at an early stage, is usually successful, so
that a careful study of these prodromes is most important. They are
generally such as denote cerebral disturbance, and, although they
should excite suspicion, are not diagnostic, except as occurring in
connection with a specific history or under suspicious circumstances.
Headache, slight failure of memory, unwonted slowness of speech,
general lassitude, and especially lack of willingness to mental
exertion, sleeplessness or excessive somnolence, attacks of momentary
giddiness, vertiginous feelings when straining at stool, yelling or in
any way disturbing the cerebral circulation, alteration of
disposition,—any of these, and, a fortiori, several of them, occurring
in a syphilitic subject, should be the immediate signal of alarm, and
lead to the examination of the optic discs, for in some cases the
eye-ground will be found altered even during the prodromic stage. Of
course if choked disc be found the diagnosis becomes practically
fixed, but the absence of choked disc is no proof that the patient is
free from cerebral syphilis. In regard to the individual prodromic
symptoms, my own experience does not lend especial importance to any
one of them, although, perhaps, headache is the most common. There is
one symptom which may occur during the prodromic stage of cerebral
syphilis, but is more frequent at a later stage—a symptom which is not
absolutely characteristic of the disease, but which, when it occurs in
a person who is not hysterical, should give rise to the strongest
suspicion. I refer to the occurrence of repeated, partial, passing
palsies. A momentary weakness of one arm, a slight drawing of the face
disappearing in a few hours, a temporary dragging of the toe, a
partial aphasia which appears and disappears, a squint which to-morrow
leaves no trace, may be due to a non-specific brain tumor, to miliary
cerebral aneurisms, or to some other non-specific affection; but in
the great majority of cases where such phenomena occur repeatedly the
patient is suffering from syphilis or hysteria.
The first type or variety of the fully-formed syphilitic meningeal
disease to which attention is here directed is that of an acute
meningitis. I am much inclined to doubt whether an acute syphilitic
meningitis can ever develop as a primary lesion—whether it must not
always be preceded by a chronic meningitis or by the formation of a
gummatous tumor; but it is very certain that acute meningitis may
develop when there have been no apparent symptoms, and may therefore
seem to be abrupt in its onset. Some years ago I saw, in consultation,
a man who in the midst of apparent health was attacked by violent
meningeal convulsions, with distinct evidences of acute meningitis. He
was apparently saved from death by very heroic venesection, but after
his return to consciousness developed very rapidly a partial specific
hemiplegia, showing that a latent gumma had {1004} probably preceded
the acute attack. On the other hand, an acute attack is liable at any
time to supervene upon a chronic syphilitic meningitis. At the
University Hospital dispensary I once diagnosed chronic cerebral
syphilis in a patient who the next day was seized with violent
delirium, with convulsions and typical evidences of acute meningitis,
and died four or five days afterward. At the autopsy an acute
meningitis was found to have been engrafted on a chronic specific
lesion of a similar character. In the case reported by Gamel,[36] in
which intense headache, fever, and delirium came on abruptly in an old
syphilitic subject and ended in general palsy and death, the symptoms
were found to depend upon an acute meningitis secondary to a large
gumma.
[Footnote 36: _Tumeurs gommeuses du Cerveau_, Inaug. Diss.,
Montpellier, 1875.]
In this connection may well be cited the observation of Molinier[37]
in which violent delirium, convulsions, and coma occurred suddenly. A
very curious case is reported by D. A. Zambaco[38] in which attacks
simulating acute meningitis occurring in a man with a cerebral
gummatous tumor appear to have been malarial. In such a case the
diagnosis of a malarial paroxysm could only be made out by the
presence of the cold stage, the transient nature of the attack, its
going off with a sweat, its periodical recurrence, and the therapeutic
effect on it of quinine.
[Footnote 37: _Revue méd. de Toulouse_, xiv. 1880, 341.]
[Footnote 38: _Des Affections nerveuses-syphilitiques_, Paris, 1862,
p. 485.]