according to Mendel, following Rokitansky's idea, a connective-tissue
disease, affecting the nerve-cells and tissues secondarily, while
Tuczek and Wernicke think that the primary disease is of the
nerve-elements (primäre Atrophie der Nervenelemente)—a diffuse
interstitial cortical encephalitis on the one hand, or a diffuse
parenchymatous cortical encephalitis on the other. There is also, in
well-marked cases, atrophy of the white substance, due, according to
general opinion of pathologists, to primary interstitial encephalitis
ending in sclerosis.
[Footnote 25: For a detailed statement of the post-mortem appearances
in general paralysis compare Spitzka's _Insanity_, pp. 218-243;
_Beiträge zur pathologischen Anatomie und zur Pathologie der Dementia
Paralytica_, von Dr. Franz Tuczek; _Die Progressive Paralyse der
Irren_, von Dr. E. Mendel; _Lehrbuch der Gehirnkrankheiten_, von Dr.
C. Wernicke, iii. pp. 536-541. Westphal's classical work is not
referred to, as his latest views and others of interest are given in a
report of a discussion by the German Association of Alienists in the
_Allgemeine Zeitschrift für Psychiatrie_, iv. 1883, pp. 634-638 and
648-654. In the third number of the _Neurol. Centralblatt_, Mendel
reports an autopsy of a patient diagnosticated to have melancholia,
who died a violent death, where he thought that he found evidence of
the early stage of general paralysis in moderate opacity of the pia
mater, with nodules as large as a pin's head in both parietal regions,
and in slight indications of diffuse interstitial inflammation of the
cortex, the blood-vessels in the frontal convolutions being
extensively filled with white blood-corpuscles.]
In the majority of cases there is pachymeningitis, often extensive and
excessive, with hemorrhages, but which may be no more than is quite
commonly found in persons dying of phthisis or chronic nephritis.
There is also, usually, leptomeningitis, with adhesions to the cortex,
especially of the anterior and antero-lateral portions, so firm that
the arachnoid cannot be removed without tearing off portions of the
brain; but it is sometimes scarcely observed, and may be no more than
is found in persons dying simply of old age. The pia may be in places
thickened, opaque, and without adhesions. Ependymitis is usual.
In the terminal stage of general paralysis there is well-marked
atrophy (with compensatory serous effusion), which is, as a rule, most
marked in the cortex of the brain, but which is of varying degrees in
its different {197} portions. Rarely there is scarcely any atrophy of
the cortex. The central portion of the brain may be of leathery
consistence, but usually shows marked sclerosis, which also may affect
its different portions and the different ganglia very differently. The
changes resulting from inflammatory, degenerative, and atrophic
processes are general and profound.
An opinion is beginning to obtain that general paralysis is primarily
a disease of the small cerebral blood-vessels, functional or
vaso-motor; and Meynert holds that the transition line between that
stage, which he considers curable, and organic disease may be
recognized clinically.
In general paralysis, as in other mental diseases, the nervous
discharge is accompanied by a greater disturbance in the structure of
the gray substance of the brain, a more extensive decompounding of it,
and consequently by a more complete exhaustion of nervous force than
in healthy mental processes. Longer periods of rest and improved
nutrition are therefore necessary to restore healthy function. In
general paralysis, as in all other mental diseases dependent upon
destructive disease of the brain, there is not only decompounding, but
decomposing and disintegrating, of the structure of the brain.
Posterior spinal sclerosis is frequently found. If alone or
predominating over sclerosis of the lateral columns of the cord, the
knee-jerk is abolished if the morbid process has gone far enough. If
descending degeneration of the lateral columns is chiefly found, and
is sufficiently advanced, the knee-jerk is increased. At least one of
these forms of sclerosis exists in the vast majority of cases.
There is also a distinctly syphilitic disease of the smaller cerebral
arteries, together with a diffuse parenchymatous and interstitial
encephalitis of syphilitic origin. At present we have no means of
differentiating it at the autopsy from general paralysis following a
subacute or chronic course, except inferentially from the presence of
other evidences of syphilis. It is not always possible, therefore, to
distinguish between syphilis and a syphilitic diathesis as the chief
factor in diffuse encephalitis.
DIAGNOSIS.—Although a well-marked case of general paralysis is
unmistakable, the diagnosis in the early stages or in obscure cases
may be extremely difficult. The varying degrees in which the various
portions of the cortex, medullary portion, and different ganglia of
the brain may be involved in the morbid process naturally give rise to
a great variety in the symptoms, mental and physical, sensory and
motor, emotional and intellectual, and in the relative preponderance
of one or another in individual cases. The usual symptoms of any form
of mental disease may for a time obscure the dementia which sooner or
later must appear in general paralysis, and which, as has already been
said, is the only mental symptom universally present in all cases.
This mental impairment must also be associated with progressive
muscular loss of power, although the relation of the two symptoms to
each other, the degree to which a given amount of the one leads to a
fair inference of a certain amount of the other, is liable to the
greatest variation, the range of which can only be learned by
observation and experience. There is a certain quality to the
dementia, as already described, which is often sufficient of itself to
establish the diagnosis with a practised physician.
{198} The early mental symptoms may simulate those of cerebral
neurasthenia, in which the patient thinks that there is decided mental
impairment, although there is no progressive dementia. The tremor in
neurasthenia is greater and more universal than in the stage of
general paralysis with which it might be confounded, and the
subjective symptoms are much more prominent.
Muscular malaise and pains throughout the body give rise to the
diagnosis of malaria or rheumatism, in which there may be loss of
power, but no ataxia or dementia.
The sclerosis may be predominating or pronounced in the basal ganglia
and bulbar nuclei, giving occasion for a hasty diagnosis of
labio-glosso-pharyngeal paralysis, until it is found that the clinical
history of that disease is not followed. In the same way, any motor or
sensory ganglia or nerve-roots may be so early implicated in the
degenerative process as to mislead the physician into giving attention
to only the local symptoms.
Once I have known the early convulsions of general paralysis in a very
self-conscious woman mistaken for hysteria, the mental impairment and
physical weakness having been overlooked on account of the prominence
of the convulsive attacks and the hysterical symptoms, which may be a
complication of any form of insanity in young and middle-aged people,
particularly women.
It is not uncommon for the attacks to so thoroughly resemble epilepsy
as to be mistaken for it, the dementia not being observed or being
supposed to be the ordinary mental deterioration generally following
epilepsy. In such cases the progressive dementia, ataxia, and muscular
weakness may advance so slowly as to entirely escape observation for a
long time, and give rise to the confident diagnosis of epilepsy for
five or six years. Epilepsy, however, arising in a vigorous,
middle-aged person without evident cause, should always suggest the
suspicion of syphilis, cerebral tumor, or general paralysis, when
careful scrutiny of all the symptoms will show where it belongs.
Embolisms, hemorrhages, cerebral effusions, more or less diffuse
encephalitis from an injury to the head, sometimes give rise to the
suspicion of general paralysis, until it is found that its
characteristic progressive symptoms do not appear, but chiefly when
the history of the case has not been definitely ascertained, or when
the usual symptoms of those conditions are not well marked.
Chronic endarteritis, arterio-sclerosis, atheroma of the cerebral
arteries may be so diffused as to simulate general paralysis,
especially in drunkards and syphilitics, but the symptoms do not
advance in the manner characteristic of that disease.
Multiple cerebro-spinal sclerosis of the descending form may be
confounded with general paralysis while the symptoms are obscure and
consist in change of character, when, indeed, organic disease can only
be suspected to be present.
Lead has been known to attack the central nervous system in such a way
as to produce an intellectual apathy and muscular weakness somewhat
resembling the early stage of the demented form of general paralysis,
but without its ataxic symptoms and its regular progress. The presence
of lead in the urine, and the marked improvement from the use of {199}
iodide of potassium, tonics, and electricity, are sufficient to
establish the diagnosis.
Chronic and persistent alcoholism is always attended with some mental
impairment, which may so resemble the dementia of general paralysis,
with marked moral perversion, mental exaltation, grand delusions,
muscular tremor, ataxic symptoms, and impaired muscular power, as to
make the diagnosis doubtful for several months, until removal of the
cause (alcohol) in the course of time causes the symptoms to so abate
as to make the real character of the disease evident.
I have once seen chronic interstitial nephritis without its usual
prominent symptoms and with mild uræmic convulsions mistaken for
general paralysis.
A tumor of the brain, if not attended with the common symptom of
vomiting, may be the cause of convulsions and headache resembling
those often seen in general paralysis. Optic neuritis or atrophy is
usual in cerebral tumor, but rare in a stage of general paralysis so
early that the diagnosis might be doubtful.
Hemorrhagic pachymeningitis also now and then simulates an obscure
case of general paralysis in the early stage, but a few weeks at most
settle any doubts in the matter.
Although diffuse cerebral syphilis is more apt to be associated with
distinctly localized symptoms than the demented form of general
paralysis, and although it is characterized by a mental apathy and
physical torpor which follow a more regular course with more definite
symptoms, resulting in a slow decay, yet there may be doubtful cases
in which the differential diagnosis is impossible, and in which
antisyphilitic treatment does not throw any light on the subject.
Syphilitic new growths, endarteritis, and meningitis may so far
improve from the use of mercury or the iodide of potassium as to end
in an apparent cure, but in those cases the symptoms are not so marked
as to make an exact diagnosis always possible. A distinct syphilitic
cachexia is presumptive evidence of syphilitic encephalitis when there
is doubt whether the syphilis is the cause or the diathesis.
Profound melancholia is not so often as varying gloom or moderate
despondency a symptom of general paralysis. When it is such, there are
developed in time the other marks of that disease, and it will only be
necessary to hold the diagnosis in reserve for their appearance. The
melancholia masks the dementia unless it is very carefully sought for,
and the tremor may be as marked in melancholia as in the early stage
of general paralysis, but more universal.
Acute mania is not uncommonly mistaken for general paralysis, when, as
often happens, the delusions are as expansive and the tremor as great
in the mania as in general paralysis; and it may be several months
before the differential diagnosis can be made with certainty. In the
presence of a high degree of maniacal excitement, with great emotional
agitation and muscular tremor, it is difficult to establish the fact
of the existence or not of dementia in doubtful cases until it is well
developed. Acute mania has been known to constitute the prodromal
period of general paralysis for a number of years.
Primary mental deterioration cannot be always differentiated from
general paralysis of the demented type in its early stage. After the
{200} age of sixty the probabilities are in favor of primary mental
deterioration in doubtful cases, but general paralysis occurs—seldom,
to be sure—up to the age of sixty-five.
Early senile dementia may simulate general paralysis of the subacute
form, but has not its clinical history. General paralysis of the
quiet, insidious type and primary mental deterioration have been
called premature senility. The three diseased conditions have certain
points of similarity, and the pathological processes involved in them
do not differ sufficiently to authorize the assumption that they are
not closely related, if not simply variations, due to age and other
causes, in one morbid process.
Finally, the mental impairment caused by the prolonged use of bromide
of potassium and hydrate of chloral has been mistaken for general
paralysis, until a critical examination unmistakably showed the
presence of the well-known symptoms of those drugs.
In examining the patient it is especially important to avoid leading
questions, as in general paralysis and in those conditions which
simulate its early stage the mind is in a condition to readily fall
into the train of thought suggested to it. The fact should be kept in
mind, too, that the symptoms in early general paralysis are so
variable as to be sometimes quite evident, and at other times not to
be got at with certainty at all or only after long and patient
examination; that they sometimes quite disappear under the influence
of complete mental and bodily rest; and that in all stages, until near
the end, such complete remissions may occur as to make the diagnosis,
independent of the history of the case, difficult if not impossible.
A gentleman once committed an offence characteristic of general
paralysis in marrying a pretty servant-girl while temporarily away
from his home. His wife, daughters, and friends saw that the act was
so contrary to his natural character that he was placed in an insane
asylum and kept there several weeks under observation for an opinion
as to his responsibility. He appeared so well in the absolute quiet
and rest that he was declared sane, tried, and sentenced to the State
prison, where he showed his marked mental impairment as soon as he was
set to work. He could not concentrate his mind sufficiently for the
simplest labor, and a couple of years later he was sent to the insane
asylum to die, a complete mental and physical wreck, in the late stage
of general paralysis.
PROGNOSIS.—The very few reported cures in so common a disease as
general paralysis, and the circumstances under which they have been
reported, lead to the suspicion that there was an error in diagnosis
or that the mistake was made of supposing a remission to be a cure, as
has often happened. The course of the disease is more rapid in men
than women, and in young persons than in the older. From the galloping
cases of a couple of months to those slowly advancing, with long
remissions, over twenty years, the average, including the prodromal
period, is probably not far from five (perhaps six) years. Collected
from asylum statistics, it is given as from two to three years. When I
am sure of the diagnosis, I generally say that the patient may die
within twenty-four hours (of paralysis of the heart, from suffocation
by an accident in an epileptic attack, from choking, from cerebral
hemorrhage or effusion, or suddenly with cerebral symptoms of which
the autopsy gives no {201} satisfactory explanation), within a short
time of intercurrent disease, especially diarrhœa or pneumonia, or
that he may live several years, as he probably will, and possibly have
a remission, during which he may lead for a while somewhat the same
kind of life as other people.
Persons presenting symptoms which can in no way be positively
distinguished from those at the beginning of the prodromal period of
general paralysis recover, but not many come under the physician's
care so early. We are not yet in a position to say whether they were
suffering from a mild, transient illness or from what would otherwise
have become serious organic disease.
TREATMENT.—Life may be prolonged in general paralysis, and usually is
prolonged, by the use of such measures as contribute to the patient's
comfort, and which in a general way have already been considered under
the head of treatment of mental disease on a previous page.
In my experience, stimulating tonics, wine, and even coffee, increase
the morbid cerebral energy of the early stage of the disease, but are
sometimes of use later. Cod-liver oil and the hypophosphites do
better, and many of the disagreeable symptoms of the period of loss of
control over the involuntary muscles are relieved by strychnia. Ergot
and the judicious use of the bromides abate the cerebral congestion.
Gastro-intestinal disorders, when not controlled by attention to diet,
require the usual treatment.
Iodide of potassium in the large or small dose, and mercury, I have
never found to benefit those cases of general paralysis with a
previous history of syphilis. On the contrary, they have proved
debilitating and harmful.
When furious excitement is not relieved by prolonged warm baths, with
cool applications to the head if possible, and quiet, chloral is of
use, and sometimes opium and its preparations.
Frequently-repeated violent convulsions, the epileptic state, are
usually at once mitigated by chloral given by the rectum; the
inhalation of nitrite of amyl is reported also to have been of use.
There are few cases in which I find that morphine does not quiet
restlessness, calm delusions, abate distressing hallucinations, and
make the patient generally more comfortable; and I give it freely,
seldom more than twice a day, often almost daily, for two or three
years. In this way it can be used in quite moderate doses. Coca also
relieves symptoms.
Rest and quiet are most important in all stages of the disease. This
can be best accomplished in a quiet private house in the country,
which can be made a virtual hospital, and next in a private asylum.
But such care is beyond the reach of the vast majority of the insane,
to whom the public asylum becomes a necessity. Wherever they are, an
orderly life is best for them, with as little irritating interference
with their ways or control of them as is possible.
If the results of treatment are in the highest degree unsatisfactory,
and consist chiefly in meeting symptoms as they come up, without hope
of permanent recovery, it is not impossible that when we can put the
patient under treatment at the very beginning of his disease, as we
can now do in pulmonary consumption, the prognosis in the former
disease may change as much for the better as it has changed in the
latter.
A general paralytic is at any time liable to congestive or maniacal
{202} attacks of short duration, and so is always, potentially, a
dangerous person. In the prodromal period the risk is small; in all
stages there will, in the majority of cases, be some warning; but in
the developed disease the only safe way is to have some responsible
person near at hand, both to prevent the patient from doing harm to
others and to save him from injuring himself, whether by intent or
through not knowing better than to wander off or fall into all sorts
of accidents. In many conditions several should be readily available,
or else the security of an asylum must be sought.
In the treatment of general paralysis by society the same rule should
obtain as in all forms of insanity—that distinct mental disease is
presumptive proof of irresponsibility, or at least of limited
responsibility; that a diseased mind means lessened intellectual power
throughout and diminished ability to choose the right and avoid the
wrong; that there are changes in circulation or nutrition, or some
unknown condition in the brain, especially in general paralysis, by
virtue of which the mental state and power of self-control vary from
time to time, and as a result of which a person seeming responsible
one day may have been quite irresponsible some previous day.
INSANITY FROM GROSS LESIONS OF THE BRAIN (tumors, new growths of all
kinds, exostoses, spicules or portions of depressed bone, embolisms,
hemorrhages, wounds, injuries, cysticerci, etc.) is attended with the
usual indications of those conditions which may determine diffuse
disorders of the brain, giving rise to any of the symptoms of the
various psycho-neuroses and cerebro-psychoses. The lowered mental and
moral tone after cerebral hemorrhages is a matter of common
observation, and after one an individual is rarely observed to be
fully himself again.
The PROGNOSIS is very unfavorable. Although there are rare cases of
improvement, the tendency is toward profound dementia.
CEREBRAL SYPHILITIC INSANITY comes either under the head of the
insanity last described or belongs to the slowly-advancing dementia
with final paralysis already referred to under the head of Diagnosis
in General Paralysis, and called by some authorities on mental disease
pseudo-paralytic dementia from syphilis.
Antisyphilitic treatment is of value in the first class of cases, and
although most of the recoveries end in relapses and incurability, the
prolonged use of iodide of potassium seems sometimes to effect a
permanent cure. It is claimed that similar treatment is followed by
the same result in the cases of dementia with paresis, but the weight
of authority, and certainly my own experience, are against that
statement.
CHRONIC ALCOHOLIC INSANITY depends upon the vascular and other changes
due to abuse of alcohol so long continued that the pathological
condition has become organic and incurable. It is commonly associated
with delusions of suspicion or persecution. It may be a purely moral
insanity, with gross beliefs rather than distinctly insane delusions,
and it rarely fails to be at least that when the persistent excessive
drinking is kept up until the age of beginning dissolution of the
brain. It then gives rise to all sorts of embarrassing complications
in regard to property, family relations, and wills. Chronic alcoholic
insanity may take the form of mild dementia, by virtue of which the
patient cannot control himself, but can be easily kept within bounds
of reasonable conduct by various degrees of restraint, from the
constant presence of a responsible {203} person to the seclusion of an
asylum. In well-marked cases this dementia is associated with muscular
weakness, tremor, and exhilaration to such an extent as to simulate
general paralysis. It is then called by some—especially French—writers
pseudo-paralytic dementia from alcohol.
The condition is susceptible of improvement by removal of the cause,
alcohol, and by a carefully-regulated life, hydropathic treatment,
etc., but complete recoveries cannot be expected.