hardly detected after the patient has had a prolonged rest and is
quiet and calm. After some emotional irritation, weariness,
sleeplessness, vaso-motor disturbances, or congestive attacks they
become very pronounced. After several weeks of absolute rest, with the
patient still at rest, it may be impossible for a time to find any
trace of mental defect or muscular deficiency until the patient has
again been put to the strain following some effort. They are very much
increased after epileptiform or apoplectiform attacks, which, however,
are uncommon so early in the case.
In the progress of the disease, as the mental impairment increases,
the reaction of the nervous system to external conditions becomes less
active, the mind weakens, the loss of flesh may be, at least in part,
regained, a great portion of the irritability and active symptoms
disappears, and as the patient grows worse he may seem for a while to
his friends to improve.
The leading symptoms of general paralysis of the insane are—(1)
vaso-motor, (2) mental, (3) physical.
The vaso-motor symptoms consist in a progressive paresis or lessened
power, which in the progress of the disease advances to complete
arterial paralysis—at first a functional disorder of impaired
innervation, and finally organic. They are marked early by rapid
changes in the cerebral circulation, a diminished arterial tension,
with occasional or frequent attacks of vertigo, dizziness, or
faintness, confusion and incoherence that may amount to a transient
dementia, localized and general elevation or {190} depression of the
bodily temperature; frequent attacks of congestion or at long
intervals, with a flushed face or transient cerebral anæmia, may be
marked by sharp emotional disturbances, fits of temper, irritability,
maniacal excitement, loss of self-control, etc., or by epileptiform
and apoplectiform seizures of various degrees of severity, with or
without temporary or transient loss of muscular power, local or of the
monoplegic, hemiplegic, or paraplegic nature, of a much less severe
character than similar attacks later in the disease, due in part also
to organic changes. The circumscribed loss of power of the vessels of
the skin leads to various functional disturbances, and finally to
paralysis, involving bed-sores, etc. Cyanosis, neuroparalytic
hyperæmia of the lungs, bladder, and intestines, cold feet, œdema of
the skin, local sweatings, etc. are final evidences of vaso-motor
paralysis. Throughout the disease, at least nearly to the end, this
vaso-motor paresis and paralysis causes marked variations in the
mental state which are too rapid to be accounted for by organic
changes.
After there is evidence of definite atrophic and degenerative disease
in the brain, as indicated by great mental impairment and muscular
paralysis, the mental and physical symptoms may be subject to great
changes, without any apparent cause but vaso-motor disturbances, and
alternating rapidly from extreme intellectual confusion and absence of
mind to a clear, even if temporary, mental state. Less extreme changes
in the condition of the mind are common.
The mental symptoms, after the disease is pronounced, consist in an
intensification of those already mentioned as characterizing the
prodromal period—in an increase in the loss of power of control over
all mental operations and in the loss of mental power, the two
symptoms making progress side by side. In the form of the disease
attended with maniacal excitement the prodromal period is usually
shorter than in the others, but may last several years. After the
prodromal period has passed the mental impairment increases, so that
the judgment, memory, power of attention and expression grow
progressively worse; and this impairment constitutes the only
characteristic mental state universally present in all stages of
general paralysis of the insane—namely, progressive dementia. The
accidental symptoms may be those belonging to any type of insanity
except logical systematized delusions. They very rarely simulate the
states of mental defect and degeneration.
If there are delusions of persecution, they are marked by a degree of
confusion or incoherence not compatible with logical inference. The
state of melancholia may change rapidly to mania, and the demented
form may at an hour's notice become the excited. Where the symptoms of
mental exaltation and depression alternate, resembling folie
circulaire, the alternation is less regular than in alternating
insanity properly speaking; hallucinations of sight and hearing and of
all the special senses are quite common, although, as a rule, rather
late symptoms, and then confused and often only partially
intelligible. There are also all sorts of illusions and delusions.
The impairment of the sense of right and wrong becomes quite marked;
the patient loses the sense of property and ownership. In no other
disease could the reported case occur of a man, to outward appearance
well, going up to a policeman and asking his assistance in rolling off
a barrel {191} of liquor which belonged to some one else, and which he
meant to appropriate. For this reason what seem to be thefts are very
common, and although by that time there is striking mental impairment,
it may not be obvious to every-day people. Almost every other moral
obliquity occurs, particularly a tendency to drunkenness and every
possible violation of the proprieties and laws regarding property and
the sexual function. It is all done, too, in such a foolish way that
the insanity would be apparent to almost any intelligent person before
whom the facts might be fully and clearly placed. There may be a
curious consciousness in the patient of the fact that something is the
matter with him, and a most extraordinary unconsciousness of what an
inordinate fool he is acting. If he can be made to see what folly he
is committing, perhaps a few moments later he is saying that he was
n-ever b-better in his life. The emotions change most rapidly, and an
adroit examiner will have his patient crying over some trifle one
moment, and another moment laughing over something equally
inconsequent. The prevailing mental state changes as rapidly as the
emotions. Violent anger, outbursts of passion, penitence, amusement do
not succeed each other more rapidly than indifference, melancholy, and
exaltation.
The suicidal idea is common in general paralytics before they become
very demented; the suicidal impulse is rarely strong enough to result
in anything more than futile attempts at self-destruction. Suicide by
deliberation is also rare, for even when it is meditated the weakened,
indecisive mind usually fails to prepare adequate plans for its
successful issue. Homicidal attempts are not to be expected as a rule,
except in the delirium of the states of maniacal excitement or in an
outburst of anger for a fancied wrong or deliberately for some
trifling reason. Even from suicide and homicide a practised physician
or attendant will easily turn the general paralytic who is not
maniacal to some amusing or silly thought. He has become credulous,
simple-minded, and easily moulded to an expert's wishes, so far as his
general conduct is concerned, and yet at any moment he is capable of a
furious mania or a violent storm of passion, which after cerebral
congestive attacks may be long and severe. Sometimes these symptoms
just described may be very pronounced at night and not especially
troublesome during the daytime. I have had patients who were
dangerous, violent, noisy, deluded at night, and entirely quiet by
day, for several months.
The mental state is of progressive impairment. The ideas flow slowly,
and there is slowness or hesitation in speech in giving utterance to
them, even to the degree of amnesic aphasia. Word-blindness occurs,
and word-deafness and the various disturbances of speech associated
with the several forms of aphasia. After the dementia is very marked
there is often a most extraordinary variation in it. The patient may
be confused, incoherent, and to appearance hardly capable of sustained
thought, but soon quite able to perform a business transaction. The
friends say of such patients, “He is crazy to-day,” or “To-day he is
sane;” and this quite independent of the marked increase in the
dementia which occurs from organic changes, epileptiform and
apoplectiform attacks, after which the advance in the mental
impairment is rapid and great. Accompanying dizzy and congestive
attacks there is a temporary dementia which may be over in an hour or
two.
{192} The patient may recall many long-past events fairly well when he
cannot find his way to the dinner-table without blundering, when he
does not know morning from afternoon, and after he is unable to dress
and undress himself without constant remindings or even actual help.
Such paralytics wander off and die of exposure, are picked up by the
police as having lost their way or as not knowing where their home is,
or fall into some fatal danger from which they have not mind enough to
extricate themselves. When the mental impairment has reached this
point the lack of mind shows itself in a lack of facial expression,
which is so characteristic of the disease that with a practised eye it
is recognized as far as the countenance can be distinctly seen; and
from this point the progress is commonly quite rapid to absolute
dementia, entire inability to form or express thoughts, too little
intellect to even attend to the daily natural wants, and a descent to
the lowest possible plane of vegetative life, and then death.
At some time or other in the history of general paralysis delusions of
grandeur, a general feeling of personal expansiveness or extreme
self-satisfaction, may be confidently looked for. In the melancholic
and hypochondriacal forms of the disease, as has already been
mentioned, they are late symptoms; in the demented type they occur
only, for the most part, near the final stage of absolute dementia;
and in the excited form they are usually found from the beginning or
at least developing from a general feeling of bien-être. They may vary
from what would pass as inordinate, silly conceit to a wildness of
delirium which stops hardly short of infinity. The patient is the
greatest financier, the handsomest man, the best runner, can out-box
the champion pugilist, can write the finest sermons. Delusions of this
degree, especially in women, are apt to refer to the reproductive
faculty or to the qualities which please the opposite sex. One man can
make a million dollars a day writing poetry; another is building
cities of solid gold; another owns all the railroads in the country,
is king over all the earth, god over God; another is running
express-trains over his bridge across the Atlantic or has a doctor who
comes to see him in a balloon. There is often a depth of vulgarity and
obscenity about the delusions which is rarely seen in other diseases.
When the grand delusions appear in the melancholic form, they are apt
to be tinged with gloom, as of a queen whose diamonds are withheld
from her, a lover who is kept from his princess bride, etc. In the
hypochondriacal form it may be a crystal liver, a silver stomach, a
brain of solid gold, etc.
Delusions of personal belittlement, called micromania, sometimes
follow or alternate with the megalomania.
Maniacal excitement is a late symptom in the demented,
hypochondriacal, and melancholic forms of general paralysis; and it
rarely occurs in them except in the final months of the disease,
unless as a direct sequence of congestive, epileptiform, or
apoplectiform attacks, and then it lasts usually from a few hours to
several days. These attacks may occur at any time without any warning
whatever, and may be attended with fury or stupor also. As a matter of
fact, they are very rare at an early stage except in the excited form
of general paralysis, of which they are a pretty constant symptom
until marked dementia has appeared, and they may continue to the end.
The fury of these maniacal attacks is of the most furious and maddest
kind, blind, the most utterly regardless of consequences {193} of any
kind of insane excitement, and without the intelligence even of the
acute maniac.
The ability to recognize his own mental state is sometimes retained by
the patient, at certain times at least, to a quite late stage in the
disease, so that he learns to call his visions hallucinations and his
strange fancies delusions. He may even agree that his illusions are
all nonsense, that the disease is in his brain. The so-called lucid
intervals are not uncommon until the final stage of absolute dementia,
when attention, memory, judgment, conception, connection of ideas,
imagination, desires, the exercise of the senses, general sensibility,
after becoming more and more imperfect are at last completely
suspended. The moral sense and finer feelings had gone long before.
Although the story of the mind's decay in general paralysis is a
comparatively short one, from a few weeks to four or five years for
the prodromal period, and then an average of two or three years for
the rest, but varying from a few weeks to a rare extreme of twenty
years, there may be at any stage of the disease, except at the very
end, a more or less complete remission lasting from a few months to
ten years. It is dangerous to say, therefore, that there is any degree
of dementia which may not be temporarily at least, or in some part,
recovered from. Most of the reported cures of general paralysis have
been at last proved to be simply remissions, which may be partial or
so complete as to leave no trace to the most practised observer. It is
not uncommon to see a remission of six months or a year or two, in
which the patient can lead a quiet life; it is seldom that he
undertakes responsibilities without bringing the remission rapidly to
an end. There are a few cases where active business has been resumed
and followed successfully for several years. But there is apt even
then to be some deterioration in character, which may amount to an
actual moral insanity. An arrest in the downward progress, so that the
symptoms remain for a considerable time without essential change, is
not very uncommon, and may occur at any stage of the disease.
The physical symptoms of general paralysis consist in impaired control
over the muscles, diminished power of co-ordinating them, followed at
once by progressive muscular enfeeblement ending in complete
paralysis.
The ataxia first shows itself in the finer muscles—of the eye, of the
fingers, of articulation. There is a little hesitancy or rather
deliberateness of speech, the voice loses its fine quality, the
intonation may be slightly nasal. Instead of contracting smoothly and
evenly as in health, the muscles show a hardly noticeable jerkiness;
an irregular fibrillary tremor is seen when they are exerted to their
utmost and held in a state of extreme tension for several moments. In
attempts to steady the handwriting the patient forms his letters
slowly, makes them larger than usual, or tries to hurry over the
letters, making them smaller. The coarser muscles show ataxic symptoms
much later. It is observed by the patient or his friends that he does
not walk off with his usual rapid gait, and the effort to co-ordinate
his muscles produces an early or unusual fatigue, which may be
associated with general muscular pain. Extreme soreness and pain,
following the course of some one or more of the main nerve-trunks, may
be most persistent and obstinate to treatment, lasting for several
years, limiting the motion of the limb, {194} sometimes beginning a
year or two before other symptoms are observed. Sooner or later,
especially after a little weariness or excitement, there are observed
at times, not constantly, indistinctness or an occasional trip in
enunciating linguals and labials, a tremor in the handwriting, a
slight unsteadiness in the gait. When the tongue is protruded as far
as possible, when the hands and arms are stretched out, when the
muscles of facial expression are exerted, in standing with feet
together with closed eyes, a decided muscular tremor and unsteadiness
are remarked. These muscular symptoms soon become constant, although
they may be so slight as to be well marked only by some unusual test,
such as prolonged use after excitement or fatigue, and the ataxia may
diminish, the gait, speech, or handwriting may improve, while muscular
power is growing progressively less.
In walking the feet are not raised as usual, the steps are shorter,
the legs are kept wider apart; turning about is accomplished in a very
deliberate way, such as to suggest an insecure feeling; movements like
dancing are impossible. Going up and down stairs is difficult; the
whole foot is rested carefully on each step, and the head and
shoulders are held stiffly, so as to maintain the balance. The
muscular movements are generally uneven and tremulous, and yet the
strength may not be so very much impaired, although perhaps available
only for short periods at a time. Even these symptoms may so improve
by a few days' quiet, or even by a night's rest, as to quite throw the
physician off his guard unless a thorough examination is made. The
patient, too, on an even floor or sidewalk may walk so as not to
attract attention, and yet in a new place, over a rough surface, or in
the attempt to perform difficult or rapid movements, exhibit striking
ataxia and feebleness of gait. In starting off with a definite purpose
he may for a short distance walk quite well, as he may do under the
influence of a glass of wine.
From this point the progress is usually rapid. The handwriting becomes
more and more tremulous, unsteady, full of omissions of letters and
words, disjointed, disconnected, and finally illegible; the
articulation more thick, stammering, hesitating, indistinct,
unintelligible; the gait staggering, shuffling, straddling, uncertain,
unsteady, even to causing frequent falls. There may be still a
considerable degree of strength for a single short effort, but the
co-ordination is so imperfect as to make it avail little. The voice,
for instance, may be loud and forcible, but the co-ordination
sufficient for only a short explosive utterance of one syllable, and
then quite an interval before the force can be concentrated for the
next. Progressive muscular paresis becomes finally absolute paralysis.
Remissions in the physical symptoms follow the same general laws as in
the mental symptoms, but are not so complete, and there may be an
arrest in their progress also.
In all stages of the disease, especially the later, there may be
almost any of the symptoms observed which occur in the various
functional and organic diseases of the nervous system. The
hyperæsthesia, local or general, may be most absolute, or the
anæsthesia so complete that acts of self-mutilation ordinarily causing
exquisite pain are performed without apparent suffering. Any motor
ganglia, any nerve, any tissue, may degenerate, giving rise to various
degrees of impairment up to total {195} destruction of function—of the
optic nerve, causing blindness; of the auditory, deafness; of the
olfactory, glosso-pharyngeal, or any of the cranial or spinal nerves.
The pupils may be of normal size. They may be of normal or sluggish
accommodation to light and distance, or there may be dilatation or
contraction of either or both pupils and no response to light or
accommodation. The pinhole pupil is not uncommon. There may be
neuro-retinitis, atrophy of the disc, neuritis, nystagmus, diplopia,
amblyopia, hemianopsia, color-blindness, ptosis, conjugate deviation
of both eyes, or paralysis of any of the ocular muscles. The paralysis
of one of the muscles of the eyeball may be one of the earliest and
most persistent symptoms. The optic neuritis or atrophy may also occur
early, but seldom appears in time to aid in a doubtful diagnosis.
Sugar has in a few cases been found in the urine; albumen is not
uncommon.
The sexual function is commonly exaggerated in the early stages, then
diminished, lost, and finally returns in the stage of absolute loss of
self-respect and self-control, although it may be impaired from the
beginning or not materially changed at first. There may be temporary
or persistent incontinence or retention of urine.
At first there is a marked loss of flesh, then a gain. As the muscles
lose in power they increase in size, with an interstitial
degeneration. The deposit of fat is sometimes enormous. In the final
stage there is emaciation.
The convulsive attacks usually are of the nature of cortical epilepsy,
or at least commonly begin as such. They are associated with and
followed by a considerable rise in temperature—from two to seven
degrees F.—and are immediately succeeded by marked increase in the
severity of the symptoms, both mental and physical, especially if the
attacks follow each other in rapid succession or last for a number of
days. They may be due to hemorrhage, embolism, or effusion, and be
marked by any or all of the usual symptoms and sequences of those
conditions, permanent or transient. General and aural vertigo are not
uncommon.
The muscular tremor before the last stages varies in different
muscles—excessive perhaps in the tongue, moderate in the fingers, and
so on. It may also seem slight as compared with the other symptoms,
or, on the other hand, be enormously exaggerated in certain groups of
muscles out of all proportion to all other indications. At the end
extreme and constant tremulousness accompanies every voluntary
movement.
Spastic paralysis, muscular tension, contractures, rigidity of the
most persistent character seem at times to be under the influence of
the will, although of cortical origin and in a certain sense
automatic, like convulsions.
The knee-jerk is changed in somewhat more than half the cases, a
little oftener exaggerated than abolished; but sometimes the reflexes
are enormously increased all over the body, so that a strong puff of
air in the face even will set the arms and legs going like a
jumping-jack. I have twice seen the patellar reflex abolished in one
leg, and so marked in the other as to seem to me exaggerated.[24] I
have also known it to disappear {196} absolutely in both legs two
weeks after it had been found to be excessively exaggerated. It also
varies under conditions of rest, fatigue, excitement, etc. Intense
pain in the joints occurs, and I have found it where the knee-jerk was
exaggerated, in one case giving rise in a physician to the delusion
that his arms and elbows had been resected. This may disappear in
time. Charcot's joint disease has been observed.
[Footnote 24: There was no evidence, and there had been no history, of
a hemiplegic attack in either case.]
In the final stages the bones are fragile and easily break;
hemorrhages under the periosteum or perichondrium arise from trifling
force or injury, giving rise to hæmatomata, the most common of which
are on parts exposed to pressure, etc., as the ear. The patient is
confined to his bed, fed like a small child, demented, hardly able to
articulate the extravagant delusions which form such a grotesque
contrast to his actual state, until the mind is as incapable of
forming or receiving ideas as of expressing thoughts; and the body is
simply a filthy, helpless mass of humanity, dying of exhaustion or
decay, unless lung gangrene, bed-sores superficial and deep,
necrobiosis, exhausting diarrhœa, pneumonia, pulmonary consumption,
perhaps asphyxia from an epileptic fit or choking, have followed
incontinence of urine and feces to the fatal end, or heart failure or
apoplexy have closed the scene.