allied to epilepsy; indeed, there are cases of cerebral syphilis in
which widespread general spasms occur similar to those of a Jacksonian
epilepsy, except that consciousness is not lost, because the nervous
discharge does not overwhelm the centres which are connected with
consciousness.[49] On the other hand, these epileptoid spasmodic cases
link themselves to those in which the local brain affection manifests
itself in contractions or persistent irregular clonic spasms.
Contractures may exist and may simulate those of descending
degeneration,[50] but in my own experience are very rare.[51]
[Footnote 49: Case, _Canada Med. and Surg. Journ._, xi. 487.]
[Footnote 50: Case, _Centralbl. Nerv. Heilk._, 1883, p. 1.]
[Footnote 51: A case of syphilitic athetosis may be found in _Lancet_,
1883, ii. 989.]
The clonic spasms of cerebral syphilis may assume a distinctly choreic
type, or may in their severity simulate those of hysteria, throwing
the body about violently.[52] It is, to my mind, misleading, and
therefore improper, to call such cases syphilitic chorea, as there is
no reason for believing that they have a direct relation with ordinary
chorea. They are the expression of an organic irritation of the
brain-cortex, and are sometimes followed by paralysis of the affected
member; in other words, the disease, progressing inward from the
brain-membrane, first irritates, and then so invades a cortical centre
as to destroy its functional power.[53]
[Footnote 52: See Allison, _Amer. Med. Journ._, 1877, 74.]
[Footnote 53: Case, _Chicago Med. Journ. and Exam._, xlvi. 21.]
Psychical Symptoms.—As already stated, apathy, somnolence, loss of
memory, and general mental failure are the most frequent and
characteristic mental symptoms of meningeal syphilis; but, as will be
shown in the next chapter, syphilis is able to produce almost any form
of insanity, and therefore mania, melancholia, erotic mania, delirium
of grandeur, etc. etc. may develop along with the ordinary
manifestation of cerebral syphilis, or may come on during an attack
which has hitherto produced only the usual symptoms. Without
attempting any exhaustive citation of cases, the following may be
alluded to.
A. Erlenmeyer reports[54] a case in which an attack of violent
headache and vomiting was followed by paralysis of the right arm and
paresis of the left leg, with some mental depression; a little later
the patient suddenly became very cheerful, and shortly afterward
manifested very distinctly delirium of grandeur with failure of
memory. Batty Tuke reports[55] a case in which, with aphasia, muscular
wasting, strabismus, {1012} and various palsies, there were delusions
and hallucinations. In the same journal[56] S. D. Williams reports a
case in which there were paroxysmal violent attacks of frontal
headache. The woman was very dirty in her habits, only ate when fed,
and existed in a state of hypochondriacal melancholy. Leiderdorf
details a case with headache, partial hemiplegia, great psychical
disturbance, irritability, change of character, marked delirium of
grandeur, epileptic attacks, and finally dementia, eventually cured by
iodide of potassium.[57] Several cases illustrating different forms of
insanity are reported by N. Manssurow.[58]
[Footnote 54: _Die luëtischen Psychosen_.]
[Footnote 55: _Journ. Ment. Sci._, Jan., 1874, p. 560.]
[Footnote 56: April, 1869.]
[Footnote 57: _Medicin Jahrbucher_, xx. 1864, p. 114.]
[Footnote 58: _Die Tertiäre Syphilis_, Wien, 1877.]
That the attacks of syphilitic insanity, like the palsies of syphilis,
may at times be temporary and fugitive, is shown by a curious case
reported by H. Hayes Newington,[59] in which, along with headache,
failure of memory, and ptosis in a syphilitic person, there was a
brief paroxysm of noisy insanity.
[Footnote 59: _Journ. Ment. Sci._, London, xix. 555.]
DIAGNOSIS.—In a diagnosis of cerebral syphilis a correct history of
the antecedents of the patients is of vital importance. Since very few
of the first manifestations of the disorder are absolutely
characteristic, whilst almost any conceivable cerebral symptoms may
arise from syphilitic disease, treatment should be at once instituted
on the appearance of any disturbance of the cerebral functions in an
infected person.
Very frequently the history of the case is defective, and not rarely
actually misleading. Patients often appear to have no suspicion of the
nature of their complaint, and will deny the possibility of syphilis,
although they confess to habitual unchastity. My own inquiries have
been so often misleading in their results that I attach but little
weight to the statements of the patient, and in private practice avoid
asking questions which might recall unpleasant memories, depending
upon the symptoms themselves for the diagnosis.
The general grounds of diagnosis have been sufficiently mapped out in
the last section, but some reiteration may be allowable. After the
exclusion of other non-specific disease, headache occurring with any
form of ocular palsy or with a history of attack of partial monoplegia
or hemiplegia, vertigo, petit mal, epileptoid convulsions, or
disturbances of consciousness, or attacks of unilateral or localized
spasms, should lead to the practical therapeutic test. Ocular palsies,
epileptic forms of attacks occurring after thirty years of age, morbid
somnolence, even when existing alone, are sufficient to put the
practitioner upon his guard. It is sometimes of vital importance that
the nature of the cephalalgia shall be recognized before the coming on
of more serious symptoms; any apparent causelessness, severity, and
persistency should arouse suspicion, to be much increased by a
tendency to nocturnal exacerbations or by the occurrence of mental
disturbance or of giddiness at the crises of the paroxysms. Not rarely
there are very early in these cases curious, almost indefinable,
disturbances of cerebral functions, which may be easily overlooked,
such as temporary and partial failures of memory, word-stumbling,
fleeting feelings of numbness or weakness, alterations of disposition.
In the absence of hysteria an indefinite and apparently disconnected
series of nerve accidents is of very urgent import. To use the words
of Hughlings-Jackson, “A random association or a random succession of
{1013} nervous symptoms is very strong warrant for a diagnosis of a
syphilitic disease of the nervous system.” Cerebral syphilis occurring
in an hysterical subject may be readily overlooked until fatal
mischief is done. When any paralysis occurs a study of the reflexes
may sometimes lead to a correct diagnosis. Thus in a hemiplegia the
reflex on the affected side in cerebral syphilis is very frequently
exaggerated, whilst in hysteria the reflexes are usually alike on both
sides. When both motion and sensation are disturbed in an organic
hemiplegia, the anæsthesia and motor paralysis occur on the same side
of the body, whilst in hysteria they are usually on opposite sides.
In all cases of doubtful diagnosis the so-called therapeutic test
should be employed, and if sixty grains of iodide of potassium per day
fail to produce iodism, for all practical purposes the person may be
considered to be a syphilitic. No less an authority than Seguin has
denied the validity of this, but I believe, myself, that some of his
reported cases were suffering from unsuspected syphilis. I do not deny
that there are rare individuals who, although untainted, can resist
the action of iodide, but in ten years' practice in large hospitals,
embracing probably some thousands of cases, I have not met with more
than one or two instances which I believed to be of such character. Of
course in making these statements I leave out of sight persons who
have by long custom become accustomed to the use of the iodide, for
although in most cases such use begets increase of susceptibility, the
contrary sometimes occurs. Of course the physician who should publicly
assert that a patient who did not respond to the iodide had syphilis
would be a great fool, but in my opinion the physician who did not act
upon such a basis would be even more culpable.
PROGNOSIS.—Cerebral meningeal syphilis varies so greatly and so
unexpectedly in its course that it is very difficult to establish
rules for predicting the future in any given case. The general laws of
prognosis in brain disease hold to some extent, but may always be
favorably modified, and patients apparently at the point of death will
frequently recover under treatment. The prognosis is not, however, as
absolutely favorable as is sometimes believed, and especially should
patients be warned of the probable recurrence of the affection even
when the symptoms have entirely disappeared. The only safety after the
restoration of health consists in an immediate re-treatment upon the
recurrence of the slightest symptom. The occurrence of a complete,
sudden hemiplegia or monoplegia is sufficient to render probable the
existence of a clot, which must be subject to the same laws as though
not secondary to a specific lesion. If a rapid decided rise of
temperature occur in an apoplectic or epileptic attack, the prognosis
becomes very grave. An epileptic paroxysm very rarely ends fatally,
although it has done so in two of my cases.
The prognosis in gummatous cerebral syphilis should always be
guardedly favorable. In the great majority of cases a more or less
incomplete recovery occurs under appropriate treatment, and I have
seen repeatedly patients who were unconscious, with urinary and fecal
incontinence, and apparently dying, recover. Nevertheless, so long as
there is any particle of gummatous inflammation in the membrane the
patient is liable to sudden congestions of the brain, which may prove
rapidly fatal, or he may die in a brief epileptic fit. On the one hand
there is an {1014} element of uncertainty in the most favorable case,
and on the other so long as there is life a positively hopeless
prognosis is not justifiable.
PATHOLOGY.—Gummatous inflammation of the brain probably always has its
starting-point in the brain-membranes, although it may be situated
within the brain: thus, I have seen the gummatous tumors spring from
the velum interpositum in the lateral ventricle. The disease most
usually attacks the base of the brain, and is especially found in the
neighborhood of the pons Varolii and the optic tract. It may, however,
locate itself upon the vault of the cranium, and in my experience has
seemed to prefer the anterior or motor regions. The mass may be well
defined and roundish, but more usually it is spread out, irregular in
shape, and more or less confluent with the substance of the brain
beneath it. It varies in size from a line to several inches in length,
and when small is prone to be multiple. The only lesion which it
resembles in gross appearance is tubercle, from which it sometimes
cannot be certainly distinguished without microscopic examination.
The large gummata have not rarely two distinct zones, the inner one of
which is drier, somewhat yellowish in color, opaque, and resembles the
region of caseous degeneration in the tubercle. The outer zone is more
pinkish and more vascular, and is semi-translucent.
On microscopic examination the most characteristic structures are
small cells, such as are found in gummatous tumors in other portions
of the body. These cells are most abundant in the inner zone, which,
indeed, may be entirely composed of them. In the centre of the tumor
they are more or less granular and atrophied; in some cases the
caseous degeneration has progressed so far that the centre of the
gumma consists of minute acicular crystals of fat. In the external or
peripheral zone of the tumor the mass may pass imperceptibly into the
normal nerve tissue, and under these circumstances it is that it
contains the spider-shaped cells or stellate bodies described by
Jastrowitch, and especially commented upon by Charcot and Gombault and
by Coyne. These are large cells containing an exaggerated nucleus and
a granular protoplasm, which continues into multiple, branching,
rigid, refracting prolongations, which prolongations are scarcely
stained by carmine. Alongside of these cells other largish cells are
often found without prolongations, but furnished with oval nuclei and
granular protoplasm. Amongst these cells will be seen the true
gummatous cells, as well as the more or less altered neuroglia and
nerve-elements. In the perivascular lymphatic sheaths in the outer
part of the gumma is usually a great abundance of small cells. The
spider-shaped cells are probably hypertrophied normal cells of the
neuroglia, and have been considered by Charcot and Gombault as
characteristic of syphilitic gummata of the brain. In a solitary
gumma, however, of considerable size from the neighborhood of the
cerebellum, studied by Coyne and Peltier, there were no stellated
cells. Coyne considers that their presence is due to their previous
existence in the normal state of the regions affected by the gumma.
Exactly what becomes of syphilitic gumma of the brain in cases of
recovery it is difficult to determine. It is certain that they become
softened and disappear more or less completely, and it is probable
that the cicatrices or the small peripheral cysts which are not rarely
found in the surfaces of the brain are sometimes remnants of gummatous
tumors. In a number of cases collected by Gros and {1015} Lancereaux
there were small areas of softened tissue or small calcareous and
caseous masses or cerebral lacunæ corresponding to the cicatrices of
softening or imperfect cysts, coincident with evidences of syphilis
elsewhere. V. Cornil also states that he has found small areas of
softening with well-established syphilitic lesions of the dura mater
and cranium, but believes that the lacunæ or cysts depend rather upon
chronic syphilitic lesions of cerebral arteries than upon gummatous
inflammation.
When a gummatous tumor comes in contact with an artery, the latter is
usually compressed and its walls undergo degeneration. The specific
arteritis may pass beyond the limit of the syphilome and extend along
the arterial wall. Not rarely there is under these circumstances a
thrombus, and if the artery be a large one secondary softening of its
distributive brain-area occurs.
TREATMENT.—The treatment of cerebral syphilis is best studied under
two heads: First, the treatment of the accidents which occur in the
course of the disease; second, the general treatment of the disease
itself.
It must be remembered that in the great majority of cases in which
death occurs in properly-treated cerebral syphilis the fatal result is
produced by an exacerbation—or, as I have termed it, an accident—of
the disease. Under these circumstances the treatment should be that
which is adapted to the relief of the same acute affection when
dependent upon other than specific cause. In a large proportion of
cases the acute outbreak takes the form either of a meningitis or else
of a brain congestion. In either instance when the symptoms are severe
free bleeding should be at once resorted to. The amount of blood taken
is of course to be proportionate to the severity of the symptoms and
the strength of the patient. I have seen life saved by the abstraction
of about a quart of blood, whilst in other cases a few ounces suffice.
Care must be, of course, taken not to mistake a simple epileptic fit
for a severe cerebral attack; but when this fit has been preceded by
severe headache and is accompanied by stupor, with marked disturbance
of the respiration, measures for immediate relief are usually
required; and if the convulsions be perpetually repeated or if there
be violent delirious excitement, the symptoms may be considered as
very urgent. In taking blood the orifice should be large, so as to
favor a rapid flow, and the bleeding be continued until a distinct
impression is made upon the pulse. In some cases which I have seen in
which the action of the heart continued to be violent after as much
blood as was deemed prudent had been taken, good results were obtained
by the hypodermic injection of three drops of the tincture of
aconite-root every half hour until the reduction of the pulse and the
free sweating indicated that the system was coming under the influence
of the cardiac sedative.
Of course, I do not mean to encourage the improper or too free use of
the lancet in these cases, but in the few fatal cases which I have
seen I have almost invariably regretted that blood had not been taken
at once very freely at the beginning of the acute attack. In most of
these cases the symptoms had progressed too far for good to be
achieved before I reached the patient. After venesection, or in feeble
cases as a substitute for it, the usual measures of relief in cerebral
congestion should be instituted. I shall not occupy space with a
discussion of these {1016} measures, as they are in no way different
from those to be employed in cases not syphilitic.
The most important part of the treatment of cerebral syphilis itself
is antisyphilitic, and the practitioner is at once forced to select
between the iodide of potassium and the mercurial preparations. In
such choice it must be remembered that even a very small amount of
syphilitic deposit in the brain may at any time cause a sudden
congestion or other acute attack, and is therefore a very dangerous
lesion. I have seen a cerebral syphilis which was manifested only by
an epileptic attack occurring once in many months, and in which after
death the affected membrane was found to be not larger than a quarter
of a dollar, and the deposit not more than an eighth of an inch in
thickness, suddenly produce a rapidly fatal congestion; and I have
known a case fast progressing toward recovery suddenly ended by the
too long continuance of the arrest of respiration during an epileptic
fit. I have, myself, no doubt of the superiority of the mercurials
over the iodide of potassium as a means of producing absorption of
gummatous exudates; and as these exudates in the brain are so very
dangerous, a mercurial course should in the majority of cases of
cerebral syphilis be instituted so soon as the patient comes under the
practitioner's care. When, however, there is a history of a recent
prolonged free use of the mercurial, or when there is marked specific
cachexia, the iodide should be chosen. Cachexia is, however, a
distinctly rare condition in cerebral syphilis, the disease usually
developing in those who have long had apparent immunity from the
constitutional disorder. In my opinion the best preparation of the
mercurial for internal use is calomel. It should be given in small
doses, one-quarter of one grain every two hours, guarded with opium
and astringents, so as to prevent as far as possible disturbance of
the bowels, and should be continued until soreness of the teeth,
sponginess of the gums, or other evidences of commencing ptyalism are
induced. After this the dose of the mercurial should be so reduced as
simply to maintain the slight impression which has been created, and
the patient should be kept under the mercurial influence for some
weeks.
A very effective method of using the mercury is by inunction, and
where the surroundings of the patient are suitable the mercurial
ointment may be substituted for the calomel. It should be applied
regularly, according to the method laid down in my treatise on
therapeutics. I have sometimes gained advantage by practising the
mercurial unction and at the same time giving large doses of iodide of
potassium internally.
After a mercurial course the iodide of potassium should always be
exhibited freely, the object being not only to overcome the natural
disease, but also to bring about the complete elimination of the
mercury from the system. There is no use in giving the iodide in small
doses; at least a drachm and a half should be administered in the
twenty-four hours, and my own custom has been to increase this to
three drachms unless evidences of iodism are produced. The compound
syrup of sarsaparilla covers the disagreeable taste of the iodide of
potassium better than any other substance of which I have knowledge.
Moreover, I am well convinced that there is some truth in the old
belief that the so-called “Woods” are of value in the treatment of
chronic syphilis. {1017} I have seen cases in which both the iodide of
potassium and the mercurials had failed to bring about the desired
relief, but in which the same alteratives, when given along with the
“Woods,” rapidly produced favorable results. The old-fashioned
Zittmann's decoction, made according to the formula of the United
States Dispensatory, may be occasionally used with very excellent
effect. But I have gradually come into the habit of substituting a
mixture of the compound fluid extract and the compound syrup of
sarsaparilla in equal proportions. The syrup itself is too feeble to
have any influence upon the system, but is here employed on account of
its flavor. A favorite method of administration is to furnish the
patient with two bottles—one containing a watery solution of the
iodide of potassium of such strength that two drops represent one
grain of the drug, and the other the sarsaparilla mixture above
mentioned. From one to two drachms of the solution of the iodide may
be administered in a tablespoonful of the sarsaparilla well diluted
after meals. When the patient has been previously mercurialized, or
there is any doubt as to the propriety of using mercurials, corrosive
sublimate in small doses may be added to the solution of the iodide,
so that one-tenth to one-fifteenth of a grain shall be given in each
dose. I have never seen especial advantage obtained by the use of the
iodides of mercury. They are no doubt effective, but are not superior
to the simpler forms of the drug.
Syphilitic Disease of the Brain-Cortex.
The psychical symptoms which are produced by syphilis are often very
pronounced in cases in which the paralysis, headache, epilepsy, and
other palpable manifestations show the presence of gross brain
lesions. In the study of syphilitic disease of the brain-membranes
sufficient has been said in regard to these psychical disturbances,
but the problem which now offers itself for solution is as to the
existence or non-existence of syphilitic insanity—_i.e._ of an
insanity produced by specific contagion without the obvious presence
of gummatous disease of the brain-membranes. Very few alienists
recognize the existence of a distinct affection entitled to be called
syphilitic insanity, and there are some who deny that insanity is ever
directly caused by syphilis. It is certain that insanity often occurs
in the syphilitic, but syphilis is abundantly joined with alcoholism,
poverty, mental distress, physical ruin, and various depressing
emotions and conditions which are well known to be active exciting
causes of mental disorder. It may well be that syphilis is in such way
an indirect cause of an insanity which under the circumstances could
not be properly styled syphilitic.
If there be disease of the brain-cortex produced directly by syphilis,
of course such disease must give rise to mental disorders; and if the
lesion be so situated as to affect the psychic and avoid the motor
regions of the brain, it will produce mental disorder without
paralysis—_i.e._ an insanity; again, if such brain disease be
widespread, involving the whole cortex, it will cause a progressive
mental disorder, accompanied by gradual loss of power in all parts of
the body, and ending in dementia with general paralysis; or, in other
words, it will produce an affection {1018} more or less closely
resembling the so-called general paralysis of the insane, or dementia
paralytica.