observation, most usually after a greater or less continuance of
prodromes such as have been mentioned, epileptic attacks have occurred
with a hemiplegia, or a monoplegia, which is almost invariably
incomplete and usually progressive; very frequently diplopia is
manifested before the epilepsy, and on careful examination is found to
be due to weakness of some of the ocular muscles. Not rarely
oculo-motor palsy is an early and pronounced symptom, and a marked
paralytic squint is very common. Along with the development of these
symptoms there is almost always distinct failure of the general health
and progressive intellectual deterioration, as shown by loss of
memory, failure of the power to fix the attention, mental
bewilderment, and perhaps aphasia. If the case convalesce under
treatment, the amelioration is gradual, the patient travelling slowly
up the road he has come down. If the case end fatally, it is usually
by a gradual sinking into complete paralysis, or the patient is
carried off by an acute inflammatory exacerbation, or, as in two of my
cases, amelioration may be rapidly occurring and a very violent
epileptic fit produce a sudden fatal asphyxia. Death from
brain-softening around the tumor is not infrequent, but a fatal
apoplectic hemorrhage is rare.
The clinical varieties of cerebral meningeal syphilis are so
polymorphic and kaleidoscopic that it is almost impossible to reduce
them to order for descriptive purposes. Fournier separates them into
the cephalic, congestive, epileptic, aphasic, mental, and paralytic,
but scarcely facilitates description by so doing. Heubner makes the
following types:
“1. Psychical disturbances, with epilepsy, incomplete paralysis
(seldom of the cranial nerves), and a final comatose condition,
usually of short duration.
“2. Genuine apoplectic attacks with succeeding hemiplegia, in
connection with peculiar somnolent conditions, occurring in
often-repeated {1005} episodes; frequently phenomena of unilateral
irritation, and generally at the same time paralyses of the cerebral
nerves.
“3. Course of the cerebral disease similar to paralytica dementia.”
In regard to these types, the latter seems to me clear and well
defined, but contains those cases which I shall discuss under the head
of Cortical Disease.
Meningeal syphilis as seen in this country does not conform rigidly
with the other asserted types, although there is this much of
agreement, that when the epilepsy is pronounced the basal cranial
nerves are not usually paralyzed, the reason of this being that
epilepsy is especially produced when the gummatous change is in the
ventricles or on the upper cortex. In basal affections the epileptoid
spells, if they occur at all, are usually of the form of petit mal;
but this rule is general, not absolute. The apoplectic somnolent form
of cerebral syphilis, for some reason, is rare in this city, and it
seems necessary to add to those of Heubner's a fourth type to which a
large proportion of our cases conform. This type I would characterize
as follows:
4. Psychical disturbance without complete epileptic convulsions,
associated with palsy of the basal nerves and often with partial
hemiplegia.
The most satisfactory way of approaching this subject is, however, to
study the important symptoms in severalty, rather than to attempt to
group them into recognizable varieties of the disease; and this method
I shall here adopt.
Headache is the most constant and usually the earliest symptom of
meningeal syphilis; but it may be absent, especially when the lesion
is located in the reflexions of the meninges which dip into the
ventricles, or when the basal gumma is small and not surrounded with
much inflammation. The length of time it may continue without the
development of other distinct symptoms is remarkable. In one case[39]
at the University Dispensary the patient affirmed that he had had it
for four years before other causes of complaint appeared. It sometimes
disappears when other manifestations develop. It varies almost
indefinitely in its type, but is, except in very rare cases, at least
so far paroxysmal as to be subject to pronounced exacerbations. In
most instances it is entirely paroxysmal; and a curious circumstance
is, that very often these paroxysms may occur only at long intervals:
such distant paroxysms are usually very severe, and are often
accompanied by dizziness, sick stomach, partial unconsciousness, or
even by more marked congestive symptoms. The pain may seem to fill the
whole cranium, may be located in a cerebral region, or fixed in a very
limited spot. Heubner asserts that when this headache can be localized
it is generally made distinctly worse by pressure at certain points,
but my own experience is hardly in accord with this. Any such soreness
plainly cannot directly depend upon the cerebral lesion, but must be a
reflex phenomenon or due to a neuritis. According to my own
experience, localized soreness indicates an affection of the bone or
of its periosteum. In many cases, especially when the headache is
persistent, there are distinct nocturnal exacerbations.
[Footnote 39: _Book Y._, p. 88, 1879.]
It will be seen that there is nothing absolutely characteristic in the
headache of cerebral syphilis; but excessive persistency, apparent
causelessness, and a tendency to nocturnal exacerbation should in any
{1006} cephalalgia excite suspicion of a specific origin—a suspicion
which is always to be increased by the occurrence of slight spells of
giddiness or by delirious mental wandering accompanying the paroxysms
of pain. When an acute inflammatory attack supervenes upon a specific
meningeal disease it is usually ushered in by a headache of
intolerable severity.
When the headache in any case is habitually very constant and severe,
the disease is probably in the dura mater or periosteum; and this
probability is much increased if the pain be local and augmented by
firm, hard pressure upon the skull over the seat of the pain.
Disorders of Sleep.—There are two antagonistic disorders of sleep,
either of which may occur in cerebral syphilis, but which have only
been present in a small proportion of the cases that I have seen.
Insomnia is more apt to be troublesome in the prodromic than in the
later stages, and is only of significance when combined with other
more characteristic symptoms. A peculiar somnolence is of much more
determinate import. It is not pathognomonic of cerebral syphilis, yet
of all the single phenomena of this disease it is the most
characteristic. Its absence is of no import in the theory of an
individual case.
As I have seen it, it occurs in two forms: In the one variety the
patient sits all day long or lies in bed in a state of semi-stupor,
indifferent to everything, but capable of being aroused, answering
questions slowly, imperfectly, and without complaint, but in an
instant dropping off again into his quietude. In the other variety the
sufferer may still be able to work, but often falls asleep while at
his tasks, and especially toward evening has an irresistible desire to
slumber, which leads him to pass, it may be, half of his time in
sleep. This state of partial sleep may precede that of the more
continuous stupor, or may pass off when an attack of hemiplegia seems
to divert the symptoms. The mental phenomena in the more severe cases
of somnolency are peculiar. The patient can be aroused—indeed in many
instances he exists in a state of torpor rather than of sleep; when
stirred up he thinks with extreme slowness, and may appear to have a
form of aphasia; yet at intervals he may be endowed with a peculiar
automatic activity, especially at night. Getting out of bed; wandering
aimlessly and seemingly without knowledge of where he is, and unable
to find his own bed; passing his excretions in a corner of the room or
in other similar place, not because he is unable to control his
bladder and bowels, but because he believes that he is in a proper
place for such act,—he seems a restless nocturnal automaton rather
than a man. In some cases the somnolent patient lies in a perpetual
stupor.
An important fact in connection with the somnolence is that it may
develop suddenly without marked premonition. Thus in a case reported
by J. A. Ormerod[40] a man who had been in good health, save only for
headache, awoke one morning in a semi-delirious condition, and for
three days slept steadily, only arousing for meals; after this there
was impairment of memory and mental faculties, but no more marked
symptoms.
[Footnote 40: _Brain_, vol. v. 260.]
Apathy and indifference are the characteristics of the somnolent
state, yet the patient will sometimes show excessive irritability when
aroused, and will at other periods complain bitterly of pain in his
head, or will groan as though suffering severely in the midst of his
stupor—at a time, {1007} too, when he is not able to recognize the
seat of the pain. I have seen a man with a vacant, apathetic face,
almost complete aphasia, persistent heaviness and stupor, arouse
himself when the stir in the ward told him that the attending
physician was present, and come forward in a dazed, highly pathetic
manner, by signs and broken utterances begging for something to
relieve his head. Heubner speaks of cases in which the irritability
was such that the patient fought vigorously when aroused; this I have
not seen.
This somnolent condition may last many weeks. T. Buzzard[41] details
the case of a man who after a specific hemiplegia lay silent and
somnolent for a month, and yet finally recovered so completely as to
win a rowing-match on the Thames. I have seen a fair degree of
recovery after a somnolence of four months' duration.
[Footnote 41: _Clinical Lectures on Dis. Nerv. Syst._, London, 1882.]
In its excessive development syphilitic stupor puts on the symptoms of
advanced brain-softening, to which it is indeed often due. Of the two
cases with fatal result of which I have notes, one at the autopsy was
found to have symmetrical purulent breaking down of the anterior
cerebral lobes; the other, softening of the right frontal and temporal
lobes, due to the pressure of a gummatous tumor, and ending in a fatal
apoplexy.
This close connection with cerebral softening explains the clinical
fact that apoplectic hemorrhage is very apt to end the life in these
cases of somnolent syphilis. But a prolonged deep stupor in persons
suffering from cerebral syphilis does not prove the existence of
extensive brain-softening, and is not incompatible with subsequent
complete recovery. As an element of prognosis it is of serious but not
of fatal import.
Paralysis.—When it is remembered that a syphilitic exudation may
appear at almost any position in the brain, that spots of encephalic
softening are a not rare result of the infection, that syphilitic
disease is a common cause of cerebral hemorrhage, it is plain that a
specific palsy may be of any conceivable variety, and affect either
the sensory, motor, or intellectual sphere. The mode of onset is as
various as the character of the palsy. The attack may be
instantaneous, sudden, or gradual. The gradual development of the
syphilitic gumma would lead us, a priori, to expect an equally gradual
development of the palsy; but experience shows that in a large
proportion of the cases the paralysis appears suddenly, with or
without the occurrence of an apoplectic or epileptic fit. Under these
circumstances it will be usually noted that the resulting palsy is
incomplete; in rare instances it may be at its worst when the patient
awakes from the apoplectic seizure, but usually it progressively
increases for a few hours, and then becomes stationary. These sudden
partial palsies probably result from an intense congestion around the
seat of disease or from stoppage of the circulation in the same
locality; whatever their mechanism may be, it is important to
distinguish them from palsies which are due to hemorrhage. I believe
this can usually be done by noting the degree of paralysis.
A suddenly-developed, complete hemiplegia or other paralysis may be
considered as in all probability either hemorrhagic or produced by a
thrombus so large that the results will be disorganization of the
brain-substance, and a future no more hopeful than that of a clot. On
the {1008} other hand, an incomplete palsy may be rationally believed
to be due to pressure or other removable cause; and this belief is
much strengthened by a gradual development. The bearing of these facts
upon prognosis it is scarcely necessary to point out.
Although the gummata may develop at almost any point, they especially
affect the base of the brain, and are prone to involve the nerves
which issue from it. Morbid exudations, not tubercular or syphilitic,
are rare in this region. Hence a rapidly but not abruptly appearing
strabismus, ptosis, dilated pupil, or any paralytic eye symptom in the
adult is usually of syphilitic nature. Syphilitic facial palsy is not
so frequent, whilst paralysis of the nerve from rheumatic and other
inflammation within its bony canal is very common. Paralysis of the
facial nerve may therefore be specific, but existing alone is of no
diagnostic value. Since syphilitic palsies about the head are in most
instances due to pressure upon the nerve-trunks, the electrical
reactions of degeneration are present in the affected muscles.
There is one peculiarity about specific palsies which has already been
alluded to as frequently present—namely, a temporary, transient,
fugitive, varying character and seat. Thus an arm may be weak to-day,
strong to-morrow, and the next day feeble again, or the recovered arm
may retain its power and a leg fail in its stead. These transient
palsies are much more apt to involve large than small brain
territories. The explanation of their largeness, fugitiveness, and
incompleteness is that they are not directly due to clots or other
structural changes, but to congestions of the brain-tissues in the
neighborhood of gummatous exudations. Squint due to direct pressure on
a nerve will remain when the accompanying monoplegia due to congestion
disappears.
Motor palsies are more frequent than sensory affections in syphilis,
but hemianæsthesia, localized anæsthetic tracts, indeed any form of
sensory paralysis, may occur. Numbness, formications, all varieties of
paræsthesia, are frequently felt in the face, body, or extremities.
Violent peripheral neuralgic pains are rare, and generally when
present denote neuritis. Huguenin, however, reports[42] a severe
trigeminal anæsthesia dolorosa, which was found, after death from
intercurrent disease, to have depended upon a small gumma pressing
upon the Gasserian ganglion. A somewhat similar case has also been
reported by Allen McLane Hamilton.[43]
[Footnote 42: _Schwiez. Corr. Blät._, 1875.]
[Footnote 43: _Alienist and Neurologist_, iv. 58.]
The special senses are liable to suffer from the invasion of their
territories by cerebral syphilis, and the resulting palsies follow
courses and have clinical histories parallel to those of the motor
sphere. The onset may be sudden or gradual, the result temporary or
permanent. Charles Mauriac[44] reports a case in which the patient was
frequently seized with sudden attacks of severe frontal pain and
complete blindness lasting from a quarter to half an hour; at other
times the same patient had spells of aphasia lasting only for one or
two minutes. I have seen two cases of nearly complete deafness
developing in a few hours in cerebral syphilis, and disappearing
abruptly after some days. Like other syphilitic palsies, therefore,
paralyses of special senses may come on suddenly or gradually, and may
occur paroxysmally.
[Footnote 44: _Loc. cit._, p. 31.]
Among the palsies of cerebral syphilis must be ranked aphasia. An
{1009} examination of recorded cases shows that syphilitic aphasia is
subject to vagaries and laws similar to those connected with other
specific cerebral palsies. It is usually a symptom of advanced
disease, but may certainly develop as one of the first evidences of
cerebral syphilis. Coming on after an apoplectic or epileptic fit, it
may be complete or incomplete: owing to the smallness of the centre
involved and the ease with which its function is held in abeyance, a
total loss of word-thought is not so decisive as to the existence of
cerebral hemorrhage as is a total motor palsy. Like hemiplegia or
monoplegia, specific aphasia is sometimes transitory and paroxysmal.
Buzzard[45] records several such cases. Mauriac[46] details a very
curious case in which a patient, after long suffering from headache,
was seized by sudden loss of power in the right hand and fingers,
lasting about ten minutes only, but recurring many times a day. After
this had continued some time the paroxysms became more completely
paralytic, and were accompanyed by loss of the power of finding words,
the height of the crises in the palsy and aphasia being simultaneously
reached. For a whole month these attacks occurred five or six times a
day, without other symptoms except headache, and then the patient
became persistently paralytic and aphasic, but finally recovered. To
describe the different forms of specific aphasia and their mechanism
of production would be to enter upon a discussion of aphasia itself—a
discussion out of place here. Suffice it to say that every conceivable
form of the disorder may be induced by syphilis.
[Footnote 45: _Loc. cit._, p. 81.]
[Footnote 46: _Aphasie et Hemiplégia droite Syphilit._, Paris, 1877.]
Owing to the centres of speech being situated in the cortical portion
of the brain, aphasia in cerebral syphilis is very frequently
associated with epilepsy. Of course right-sided palsy and aphasia are
united in syphilitic as in other disorders. If, however, the
statistics given by Tanowsky[47] be reliable, syphilitic aphasia is
associated with left-sided hemiplegia in a most extraordinarily large
proportion. Thus in 53 cases collected by Tanowsky, 18 times was there
right-sided hemiplegia, and 14 times left-sided hemiplegia, the other
cases being not at all hemiplegic. Judging from the autopsy on a case
reported in Mauriac's brochure, this concurrence of left-sided
paralysis and aphasia depends partly upon the great frequency of
multiple brain lesions in syphilis, and partly upon the habitual
involvement of large territories of the gray matter secondarily to
diseased membrane. An important practical deduction is that the
conjoint existence of left hemiplegia and aphasia is almost diagnostic
of cerebral syphilis.
[Footnote 47: _L'Aphasie syphilitique_.]
Probably amongst the palsies may be considered the disturbances of the
renal functions, which are only rarely met with in cerebral syphilis,
and which are probably usually dependent upon the specific exudation
pressing upon the vaso-motor centres in the medulla. Fournier speaks
of having notes of six cases in which polyuria with its accompaniment,
polydipsia, was present, and details a case in which the specific
growth was found in the floor of the fourth ventricle. Cases have been
reported of true saccharine diabetes due to cerebral syphilis,[48] and
I can add to these an observation of my own. The symptoms, which
occurred in a {1010} man of middle age, with a distinct specific
history, were headache, nearly complete hemiplegia, and mental
failure, associated with the passage of comparatively small quantities
of a urine so highly saccharine as to be really a syrup. Under the
influence of the iodide of potassium the sugar in a few weeks
disappeared from the urine.
[Footnote 48: Consult Servantié, _Des Rapports du Diabète et de la
Syphilis_, Paris, Thèse, 1876; also, case reported by L. Putzel, _New
York Med. Record_, xxv. 450.]
Epilepsy.—Epileptic attacks are a very common symptom of meningeal
syphilis, and are of great diagnostic value. The occurrence in an
adult of an epileptic attack or of an apoplectic fit, or of a
hemiplegia after a history of intense and protracted headache, should
always excite grave suspicion.
Before I had read Fournier's work on _Nervous Syphilis_ I taught that
an epilepsy appearing after thirty years of age was very rarely, if
ever, essential epilepsy, and unless alcoholism, uræmic poison, or
other adequate cause could be found was in nine cases out of ten
specific; and I therefore quote with satisfaction Fournier's words:
“L'épilepsie vraie, ne fait jamais son premier dêbut à l'âge adulte, à
l'âge mûr. Si un homme adulte, au dessus de 30, 35, à 40 ans, vient, à
être pris pour la première fois d'une crise épileptique, et cela dans
la cours d'une bonne santé apparente, il y a, je vous le répète, hui
ou neuf chances sur dix pour que cette épilepsie soit d'origine
syphilitique.”
Syphilitic epilepsy may occur either in the form of petit mal or of
haut mal, and in either case may take on the exact characters and
sequence of phenomena which belong to the so-called idiopathic or
essential epilepsy. The momentary loss of consciousness of petit mal
will usually, however, be found to be associated with attacks in
which, although voluntary power is suspended, memory recalls what has
happened during the paroxysm—attacks, therefore, which simulate those
of hysteria, and which may lead to an error of diagnosis.
Even in the fully-developed type of the convulsions the aura is only
rarely present. Its absence is not, however, of diagnostic value,
because it is frequently not present in essential epilepsy, and it may
be pronounced in the specific disease. It is said that when in an
individual case the aura has once appeared the same type or form of
approach of the convulsion is thereafter rigidly adhered to. The aura
is sometimes bizarre: a severe pain in the foot, a localized cramp, a
peculiar sensation, indescribable and unreal in its feeling, may be
the first warning of the attack. An aura may affect a special sense.
Thus, I have at present a patient whose attacks begin with blindness.
In many, perhaps most, cases of specific convulsions, instead of a
paroxysm of essential epilepsy being closely simulated, the movements
are in the onset, or more rarely throughout the paroxysm, unilateral;
indeed, they may be confined to one extremity. This restriction of
movement has been held to be almost characteristic of syphilitic
epilepsy, but it is not so. Whatever diagnostic significance such
restriction of the convulsion has is simply to indicate that the fit
is due to a cortical organic lesion of some kind. Tumors, scleroses,
and other organic lesions of the brain-cortex are as prone to cause
unilateral or monoplegic epilepsy when they are not specific as when
they are due to syphilis.
Sometimes an epilepsy dependent upon a specific lesion implicating the
brain-cortex may be replaced by a spasm which is more or less local
and is not attended with any loss of consciousness. Thus, in a case
now {1011} convalescent in the University Hospital, a man aged about
thirty-five offered a history of repeated epileptic convulsions, but
at the time of his entrance into the hospital, instead of epileptic
attacks, there was a painless tic. The spasms, which were clonic and
occurred very many times a day, sometimes every five minutes, were
very violent, and mostly confined to the left facial nerve
distribution. The trigeminus was never affected, but in the severer
paroxysms the left hypoglossal and spinal accessory nerves were
profoundly implicated in all of their branches. Once, fatal asphyxia
from recurrent laryngeal spasm of the glottis was apparently averted
only by the free inhalation of the nitrite of amyl. The sole other
symptom was headache, but the specific history was clear and the
effect of antisyphilitic remedies rapid and pronounced.