THE PSYCHOSES WITH HUNTINGTON'S CHOREA, BRAIN TUMOR AND OTHER BRAIN OR
NERVOUS DISEASES
Huntington's chorea is said to have been referred to first by C. O.
Waters of Franklin, N. Y., in Dunglison's "Practice of Medicine" in
1842. An article on the subject by Irving W. Lyon also appeared in
the _American Medical Times_ in 1863. The name by which the disease
is now generally known was the result of an elaborate description of
its symptomatology by George Huntington in the _Medical and Surgical
Reporter_ in 1872. He particularly called attention to the fact that
it is hereditary in origin, occurs in adult life, is associated
with suicidal tendencies and often exhibits mental symptoms. On
the important subject of heredity Huntington made the following
observation: "If one or both of the parents have shown manifestations
of the disease, and more especially when these manifestations have been
of a serious nature, one or more of the offspring almost invariably
suffer from the disease if they live to adult life; and if by any
chance these children get through life without it, the thread is broken
and the grandchildren or great grandchildren may rest assured that they
are free from the disease. Unstable and whimsical as the disease may
be in other respects, in this it is firm; it never skips a generation
to manifest itself in another; as soon as it has yielded its claims,
it never regains them." A well known monograph on the subject by Osler
appeared in 1894.
McCarthy[189] refers to the mental condition associated with this
disease as "a severe and gradually progressive deterioration,
ultimately ending in absolute dementia. In some cases the mental defect
is noted from the onset of the symptoms, in others the mentality may
remain unimpaired for years. Mental deterioration is the rule, and it
is associated with a loss of memory and a tendency to self-destruction
which gradually develops. When the mental degeneration is well marked,
outbreaks of violence are sometimes noted. In one of the writer's
patients, as the disease progressed, the clinical picture of paresis
was presented. The chronic delusional state is more often noted than
would be inferred from Huntington's description." Hamilton,[190] who
made a clinical study of a considerable series of cases in 1907,
expressed the opinion that mental deterioration occurs in the majority
of instances before the onset of choreiform symptoms. He found a
special tendency to deterioration in the cases appearing early in
life, while irritability and delusional ideas were more often observed
in those developing in later years. Delusions of persecution and
deterioration, however, were symptoms more or less common to both
groups. Diefendorf,[191] in a study of twenty-eight cases in 1908,
called attention particularly to the irritability with occasional
outbursts of violence as well as attacks of despondency. He emphasizes
emotional deterioration and indifference.
Kraepelin[192] also refers to the fact that the mental symptoms may
precede the choreiform manifestations in appearance, sometimes by a
number of years. The patients become forgetful, defective in judgment,
somewhat dull, show a poverty of thought and an incapacity for orderly
activities. Generally there is an emotional depression, often with
irritability and more rarely euphoric symptoms. Delusions gradually
develop. These are of a persecutory nature, although ideas of grandeur
appear at times. Suicidal tendencies are common. Disturbances of
perception and memory may be very pronounced. The relation of the
patient to his environment becomes very much confused. In some cases,
on the other hand, the mental symptoms are not very striking. Anxious
states, outbursts of anger or emotional excitements may appear at
times. Appetite and sleep are often interfered with. The pathology of
this disease is not characteristic. There may be a chronic meningitis
or extensive atrophies. The cells of the third layer of the cortex,
according to Kraepelin, are decreased in number with an increase of
glia nuclei. The remaining cells are shrunken with deeply staining
processes, and there is a considerable loss of tangential fibres.
Sclerotic changes with thickened walls are noted in the blood vessels.
Hyaline degeneration and miliary hemorrhages have been observed,
although Nissl and Alzheimer found no vascular lesions worthy of note.
The cortical changes, according to Räcke, are more pronounced in the
central convolutions, being much less conspicuous in the frontal and
occipital regions. Alzheimer found the corpus striatum particularly
involved. Here he noted a striking cell loss, with glia proliferation
but no vascular changes. D'Ormea, according to Kraepelin, traced the
disease through five generations in one family and Browning went as far
back as two hundred years in another.
The observations on the subject of Huntington's chorea in the
statistical manual of the American Psychiatric Association are as
follows:—
"Mental symptoms are a constant accompaniment of this form of chorea
and as a rule become more marked as the disease advances. Although the
disease is regarded as being hereditary in nature, a diagnosis can be
made on the clinical picture in the absence of a family history.
"The chief mental symptoms are those of mental inertia and an emotional
change, either apathy and silliness or a depressive irritable reaction
with a tendency to passionate outbursts. As the disease progresses the
memory is affected to some extent, but the patient's ability to recall
past events is often found to be surprisingly well preserved when the
disinclination to cooperate and give information can be overcome.
Likewise the orientation is well retained even when the patient
appears very apathetic and listless. Suspicions and paranoid ideas are
prominent in some cases."
Statistical reports from American institutions show that comparatively
few cases of Huntington's chorea are committed. In 49,640 first
admissions to the New York state hospitals only forty-eight, or .09 per
cent, were diagnosed as Huntington's chorea during a period of eight
years. The admission rate to the Massachusetts hospitals during 1919
was exactly the same. In twenty-one hospitals in fourteen other states
twenty-four cases (.13 per cent) in 18,336 admissions were reported as
Huntington's chorea. There were only seventy-five cases (.1 per cent)
in 70,987 admissions to forty-eight hospitals in sixteen different
states.
_Psychoses with Brain Tumor_
Brain tumors are more common perhaps than is generally understood.
Cushing[193] shows that they were found in fifty-five, or 1.7 per cent,
of 3,150 autopsies at the Johns Hopkins Hospital. He refers to Siedel,
who observed them in 1.25 per cent of his cases in Munich and states
that Blackburn found them in about two per cent of 1,642 autopsies at
the St. Elizabeths Hospital in Washington. He also quotes Bruns as
saying that two per cent of all neurological cases show intracranial
growths. In the first twenty-five hundred surgical conditions admitted
to the Peter Bent Brigham Hospital in Boston eight per cent were
diagnosed as brain tumor. Cushing found that 66.6 per cent of 130
carefully studied growths were gliomata. Nearly four per cent were
endotheliomas. In another series of seventy cases he found twenty-seven
gliomas (38.5 per cent), seventeen adenomas (twenty-four per cent),
seven endotheliomas (ten per cent), five interpeduncular and mixed
growths (seven per cent), and other forms in smaller percentages. Many
of the endotheliomas have undoubtedly been included in the past with
the sarcomas. This may also be said of gliomas.
According to Cushing, growths in the brain may give rise to no
disturbance whatever, show well defined focal signs, occasion only
general manifestations, or have both general and focal symptoms,
depending on the location of the neoplasm. General symptoms may be
briefly summarized as follows:—headache, vomiting, choked disc,
vertigo, drowsiness, convulsions, disturbances of the pulse rate,
respiration and temperature, as well as mental disorders. The focal
signs depend wholly on the site of the growth. Cushing mentions the
following symptom complex as resulting from lesions of the frontal
lobes:—"Indifference, unpunctuality, mental enfeeblement, loss of
memory and power of attention, change in disposition with more or less
marked irritability or taciturnity or obstinacy or jocularity, etc.,
often a rambling speech, lack of realization of the illness, and change
in the general conduct of life with habits of untidiness. These, in
greater or less degree, characterize most of the cases, although it is
often astonishing to find how inconspicuous the symptoms may be with a
very extensive growth. They may often be of rather abrupt onset and not
until the situation of the lesion is definitely disclosed and careful
interrogation made into the patient's previous mental state is it
possible to learn that in all probability some mental alteration has
been of long standing."
Bruns did not find psychoses associated very often with frontal
lesions. Jacobi, however, in reviewing the literature of growths in
that region, found mental symptoms in forty-nine per cent. Schuster
observed them in from fifty to sixty per cent of all brain tumors.
Redlich[194] described mental conditions as being either incidental and
not related to the growth, or definitely caused by it, and was even of
the opinion that the neoplasm could in some instances be the result of
a psychosis. Two of Redlich's patients, moderately alcoholic, showed a
typical Korsakow syndrome. He refers to the fact that in cases reported
by Oppenheim, Friedrich and Fürstner, "Witzelsucht," or the tendency to
joke, disappeared after growths were removed from the frontal region.
A patient of Begerthal, who had hallucinations, delusions and somatic
symptoms, recovered after a tubercle was excised from the paracentral
lobule. A case of Friedrich's which showed an alteration of the
personality, erotic symptoms, sudden explosive laughter, poor memory,
etc., recovered after a sarcoma was removed from the right frontal
lobe. A patient of Thoma's after three attacks of mental depression
showed a gliosarcoma in the occipital lobe at autopsy. Schuster, Bruns
and Schönthal have reported cases of brain tumor with hysterical
manifestations.
Redlich described the psychoses associated with cerebral growths
as being epileptiform in character and origin and resembling
post-epileptic psychoses in their symptomatology, with irritability,
excitement or violence, confusion, delirium and hallucinations, often
followed by partial amnesia. Epileptic manifestations may occur in the
form of equivalents during the development of the growth. Bernhardt and
Oppenheim have called attention to episodes of vertigo, irritability,
excitement, clouding and occasional delirium with amnesia following
intense paroxysms of headache. These attacks also strongly suggest
the characteristics of epileptic psychoses. Nothnagel, Bernhardt,
Oppenheim, Schuster, Ziehen and others attribute the mental symptoms
associated with brain tumor to increased intracranial pressure
producing an anaemia. Klippel, Maillard, Vigouroux, Kaplan and others
believe that they are due to toxins originating in the growth. This
view is based largely on the appearance of psychoses similar to the
Korsakow syndrome. Knapp in 1906 called attention to the prominence of
mental symptoms in growths occurring in the anterior portion of the
corpus callosum. These may be associated with intellectual defects,
apraxia, speech disturbances and stupor. Gianelli found mental
disturbances in 209 of 318 cases examined.
Kraepelin[195] attributes the mental symptoms of growths to an injury
of the brain structure, changes in intracranial pressure, circulatory
disturbances, and the absorption of toxic substances. A growth of
considerable size but of slow development may permit of a readjustment
of pressure, etc., and show few symptoms. On the other hand, a
small neoplasm on account of its site or rapidity of growth may be
accompanied by profound mental disturbances resulting from chemical
irritation, obstruction of the aqueduct of Sylvius, or circulatory
interferences. Kraepelin quotes Schuster (1902) as finding psychotic
symptoms in all cases of growths in the corpus callosum, in two-thirds
of those of the hypophysis, in one-third of those of the cerebellum and
in one-fourth of the cases with involvement of the brain stem. These he
looks upon as pressure symptoms except in the case of the callosal
neoplasms. Schuster was of the opinion that growths in the cortex
usually lead to actual psychoses and those in the deeper areas to
dementia. He found a general mental deterioration in 423 out of a total
of 775 cases of brain tumor. The patients were indolent, inattentive,
clumsy, forgetful, dull, tired easily and lost more and more their
capacity and inclination for sustained exertion. Thought, decision and
mental processes generally, required an unusual amount of effort. The
patients usually became somewhat confused and disoriented in regard to
time, place and person, as well as incoherent in speech. In many cases
there is a marked memory disturbance with a tendency to fabrication
suggesting Korsakow's psychosis. Delirious states with hallucinations
sometimes accompany growths in the posterior lobes. Kraepelin has also
observed hallucinations in cases with tumor of the cerebellum. Many
develop hypochondriacal ideas, others have delusions of persecution or
self-accusation and suicidal tendencies. Rarely there are delusions of
grandeur. The mood is usually anxious, depressed and at times
irritable or apathetic. Occasionally the patients may, on the other
hand, be cheerful in spite of the hopeless condition they are in.
They may even show distractibility, flight of ideas, volubility and
excitement. There is more often a childish elation with a tendency to
joking and facetiousness. Schuster found this more common in frontal
involvements. Kraepelin also called attention to restlessness and
excitements often leading to violence. This may alternate with mental
dulness and cataleptic states. The patients may repeat words and make
meaningless response to questions, strongly suggesting katatonia.
Mental dulness becomes more and more marked, however, even reaching
a stuporous stage. To this is added, according to the location of
the growth, focal symptoms of various kinds—headache, disturbance
of vision, seizures, paralyses, aphasia, agraphia, articulatory
disturbance, ataxia, etc. Special symptoms arise where psychogenic
factors play a part,—excitements with paralyses or disturbance of
perception, etc. Hysterical stigmata may appear. Cases with growths in
the frontal region occasionally simulate general paresis but should be
distinguished without difficulty.
The Association's statistical manual has the following to say of
psychoses with brain tumor:—
"A large majority of brain tumor cases show definite mental symptoms.
Most frequent are mental dullness, somnolence, hebetude, slowness in
thinking, memory failure, irritability and depression, although a
tendency to facetiousness is sometimes observed. Episodes of confusion
with hallucinations are common; some cases express suspicions and
paranoid ideas.
"The diagnosis must rest in most cases on the neurological symptoms,
and these will depend on the location, size and rate of growth of
the tumor. Certain general physical symptoms due to an increased
intracranial pressure are present in most cases, viz.: headache,
dizziness, vomiting, slowing of the pulse, choked disc and interlacing
of the color fields."
The number of cases reaching hospitals for mental diseases is, of
course, small. In 49,640 first admissions to the New York state
hospitals in eight years there were sixty-seven cases (.14 per cent)
of psychoses with brain tumor. In 18,336 admissions to twenty-one
hospitals in fourteen other states there were eighteen cases (.09 per
cent) diagnosed as psychoses with brain tumor. There were ninety-three
cases (.13 per cent) in 70,987 first admissions to forty-eight
hospitals for mental diseases in sixteen different states.
_Psychoses with Other Brain or Nervous Diseases_
Cerebral hemorrhages, thrombosis and embolism are more or less
intimately associated etiologically, pathologically and clinically.
They all bear a rather definite relation to the general question of
arteriosclerosis and may all lead to cerebral softening. Apoplexy is
a term which was employed by Aristophanes, Demosthenes and Sophocles
and has been in general use for centuries. It was known to Chaucer and
was referred to in Shakespeare's works ("Henry IV"). It was studied
very elaborately by Sydenham and many other early writers. Charcot and
Bouchard in 1864 called attention to the relation existing between
miliary aneurysms of the cerebral vessels and hemorrhages. In a study
of the cerebral vascular lesions at the University College Hospital,
London, Jones (_Brain_, 1905) found records of one hundred and sixty
cases occurring during a period of sixty-five years. Of these, 123
showed hemorrhages; twenty-four, thrombosis; and thirteen were due to
embolism.
Thomas[196] states that: "The symptoms following acute vascular lesions
of the brain, whether the process be the rupture of a vessel or its
occlusion, are in many respects identical; and clinically it is often
impossible to determine which process has been effective." He calls
attention to the fact that in thrombosis the final closure of a vessel
may occur suddenly and the symptoms develop with great rapidity. On
the other hand, the rupture of a vessel may mean the escape of only a
small quantity of blood and after an embolism the circulation is not
always stopped immediately. In an analysis of 401 apoplectic attacks
Thomas found no loss of consciousness in 202 cases, although it was
interrupted or markedly disturbed in 199. Jones found a complete loss
of consciousness in 47.7 per cent of 201 cases of cerebral embolism
and a partial disturbance in sixty per cent. He reported consciousness
affected in seventy-five per cent of his cases of cerebral hemorrhage
and in 45.5 per cent of those of thrombosis. When it occurs it is
usually not the initial symptom in his experience, being preceded by
headache, vertigo, weakness in certain parts of the body, etc. An
analysis of the cases of embolism reported by Virchow, however, showed
a sudden loss of consciousness as the initial symptom to be the general
rule. Gowers is of the opinion that an initial softening is a more
common occurrence than hemorrhage.
In the young, apoplectic attacks are usually due to cerebral softening,
thrombosis following acute disease or embolism resulting from
endocarditis. Between the ages of twenty and forty apoplexies usually
mean syphilitic thrombosis. In the later decades of life, either
hemorrhage, thrombosis, embolism or softening may occur. Thomas[197]
collected from various hospitals, statistics of 840 cases. Of these,
499 showed hemorrhages and 341 softenings. He is of the opinion that
the presence of premonitory symptoms for some days indicate thrombosis,
while shorter prodromal periods point to a hemorrhage. Rapidly
developing coma suggests hemorrhage, while a widespread paralysis
without much disturbance of consciousness is more common in thrombosis.
A marked fall of temperature and rise of blood pressure as a rule
means a hemorrhage. Repeated convulsions are more often associated
with softening or embolism. If the symptoms indicate a capsular lesion
it favors hemorrhage, and if of a cortical type, softenings are
more likely. A positive Wassermann reaction suggests thrombosis or
softening. The presence of endocarditis with heart murmurs points to
embolism. Thomas finds that, while the patient may recover from either
of these conditions without apparent intellectual defect, he is liable
to be petulant, emotional, depressed and tire easily.
In psychoses following hemorrhage, thrombosis and embolism
Kraepelin[198] as a rule finds very little relation between the nature
of the lesion in question and the symptoms to be expected. Immediately
following the seizure the patients become dull, clouded, confused
and disoriented, and peculiar in their behavior. This is followed by
an active excitement with loud cries, resistiveness and struggling.
These acute disturbances usually subside, leaving, however, evidences
of the arteriosclerosis or syphilitic endarteritis which caused the
hemorrhage or thrombosis. Embolism may leave an apparently permanent
mental deterioration with aphasic and paraphasic manifestations which
often entirely clear up. In lesions of younger persons due to syphilis,
mental enfeeblement may follow.
Our knowledge of the psychoses accompanying paralysis agitans is very
inadequate. The disease was first fully described by Parkinson in an
English publication in 1817, although, according to Camp, similar
cases were reported by Schwarz in 1766. The etiology of this condition
is unknown and the pathology is not at all definite. It seems to be
the rather general opinion of neurologists that mental disturbances
are quite rare in Parkinson's Disease. Camp,[199] for instance, has
the following to say on this subject:—"Mental conditions have also
been described, but usually the patient's mind is entirely clear. In
the very old the changes incident to senility, such as irritability,
childishness, etc., insomnia and memory changes, might be expected and
may require special treatment. Often these patients are emotionally
unstable and spells of forced weeping or laughter occur." Krafft-Ebing
refers to mental weakness in paralysis agitans and speaks of the
frequency of melancholia with hallucinations and suicidal impulses
occurring intermittently and appearing with exacerbations of the
disease. He speaks of premature senility as playing the most important
etiological rôle. McCarthy[200] expresses the opinion that: "Beyond
a tendency on the part of some patients to adopt a whining and
complaining manner, the mind remains very clear; in fact, the good
nature and complaisance of most of the patients, in spite of the
severity of the symptoms, is a matter of common observation. Dementia
may, however, complicate a case of the disease." On the other hand,
Parant, a French writer who made an elaborate study of this subject in
1883, described three distinct varieties of mental disturbance observed
by him. In the milder cases he found changes in the personality.
This is shown by irritability, egotism, restlessness, suspicion,
undue sensitiveness regarding their disease, mild persecutory ideas,
tendencies to depression, indifference and apathy. The second class
of cases described included mental deterioration with difficulty
of thought, loss of memory, etc. The third group includes definite
psychoses characterized generally by depressions with or without
hallucinations and delusions. Hallucinations of sight are said to be
common. Delusions of persecution are prominent and hypochondriacal
and somatic ideas frequently occur. Suicidal tendencies are very
pronounced. According to Ball, these episodes come and go "with the
aggravation of the sensory symptoms, and they seem to disappear when
the tremor decreases or ceases entirely." The usual tendency in these
cases, as shown by Parant, is to terminate in complete deterioration.
Of the inflammatory conditions of the meninges Kraepelin[201] makes
special reference to mental disturbances associated with tuberculosis.
The patient is depressed, anxious, irritable and apathetic, often with
the first appearance of the disease. Dulness and memory disturbances
become more and more apparent. The patient soon becomes clouded and
disoriented, confused and delirious. Occasionally hallucinations
appear. The disturbance of consciousness becomes more and more marked.
The patient becomes incoherent, restless, noisy and often violent.
The excitement may reach the stage of an actual mania with delirious
confusion. Sometimes the symptoms are strongly suggestive of katatonia.
In alcoholics a condition very similar to delirium tremens develops,
terminating as a rule in stupor and coma. Speech disturbance, aphasia,
convulsions, hyperesthesia or muscular weakness may be observed in
such cases. Other forms of meningitis are quite similar but more rapid
in development and of shorter duration. In some instances, as after
epidemic cerebrospinal meningitis, states of mental enfeeblement may
follow the disease.
It must be admitted that our information on the subject of multiple
sclerosis is far from being complete. In a discussion of the mental
symptoms accompanying this condition, Henderson[202] expressed the
following views:—"Cases of disseminated sclerosis which present
definite, well marked psychoses are extremely rare. When mental
symptoms do occur, they usually come on when the condition is well
advanced, the most common symptoms are mild euphoria, labile mood,
apathy and dullness, and a slightly defective memory. In some
cases, however, depression has been described as the outstanding
feature, while hallucinations of sight and hearing are not uncommon
accompaniments. In certain cases the mental symptoms may come on
early, and these are usually of excessive severity and are rapidly
followed by complete dementia." Dunlap has described cases associated
with general paresis and showing the characteristic lesions of both
diseases at autopsy. According to Kraepelin[203] mental disturbances
sometimes appear before physical symptoms are observed. These take the
form of depression, anxiety, fear, with occasional deliria, hysterical
manifestations, emotional dulness, variable moods and a marked
irritability. Later in the disease more marked euphoric or depressive
tendencies appear, with excitements and confusional states. Delusions
of a persecutory nature, or ideas of grandeur may be observed.
Hallucinations are infrequent. According to Kraepelin, from ten to
thirty per cent of the cases terminate in a general mental enfeeblement
which is not usually of an advanced degree. He also describes a lobar
cortical sclerosis with much more marked mental disturbances suggesting
dementia praecox.
Various mental conditions have been attributed to tabes. Sachs[204]
speaks of depressions and neurasthenic conditions with irritability as
a special symptom. He has observed paranoid states and manic attacks,
sometimes with periods of "transitory dementia" with or without
aphasia. He also expresses the opinion that tabetics may develop
all of the symptoms of general paresis, although he says that the
coexistence of the two diseases is rare. Kraepelin[205] speaks of
milder forms of psychoses characterized by uncertainty of memory,
fatigability and emotional instability. Many cases exhibit a hopeless,
gloomy attitude with depression and fears, or they may be surly,
irritable and quarrelsome. Others show a surprisingly good humor.
The emotional disturbances often suggest general paresis. Kraepelin,
however, describes the characteristic psychosis of locomotor ataxia
as assuming a paranoid form and quotes Meyer as reporting paranoic
conditions in twenty-six tabetics and depressions of various types
in fourteen. He also speaks of hallucinatory excitements resembling
alcoholic conditions. These are characterized by a sudden anxiety
and restlessness with hallucinations of both hearing and vision. The
patients complain of poisoning and sensations of electricity, but
are cheerful in mood and well oriented. This condition may last for
weeks or months, ending in a sudden recovery, often with relapses.
Shorter hallucinatory delirious states resembling crises are also
referred to by Kraepelin. More chronic conditions are noted, with
hallucinations, persecutory delusions and ideas of grandeur. Delirium
tremens, manic-depressive attacks, katatonia or senile psychoses may be
associated with tabes.
The literature of medicine contains many references to acute chorea. It
was referred to, according to Paton, by Plat as early as 1614 and was
discussed by Sydenham at some length. Wharton Sinkler, in describing
chorea in Pepper's "System of Medicine" in 1886, made the following
interesting remarks on the mental changes involved:—"The child is
irritable and feverish, cries and laughs readily, or is sullen and
morose. Sometimes he is violent to those about him but this is rare.
Intellectually the patient suffers somewhat. He is not able to study
as before, and the memory may be impaired. Sometimes there is a mild
form of dementia." Burr[206] divides these conditions into four
groups:—"First (and this includes the vast majority), patients in
whom there is peevishness, fretfulness, some loss of the power of
fixing the attention, and a slight loss of the moral sense shown by
disobedience and selfishness. Second, those showing in addition to
the above symptoms, night terrors, and transitory, visual, auditory,
or other hallucinations. Third, those with distinct delirium, wild
or mild, accompanied with fever. Fourth (and this group is very
small when we remember how common chorea is), those showing stupor,
or rather stupidity, and an acute dementia, which may follow the
condition described under three, or appear without any preceding
mental symptoms at all severe, and which is usually accompanied with
trouble on articulation not caused by choreic movements of the lips
and tongue, but the result of mental hebetude." White[207] refers to
the irritability and emotional instability of choreics and describes a
psychosis in "chorea insaniens" characterized by an acute confusion,
sometimes of a violent type with hallucinations, or a paranoic
condition with delusions of persecution. This may develop into a
stuporous state. Kraepelin describes the psychotic manifestations of
acute chorea as forms of delirium due to infection with characteristic
states of clouding, confusion, etc. Wechsler has expressed similar
views.
Encephalitis lethargica is a disease which has received a great deal
of attention during the last few years. The term was first applied
by von Economo[208] to a series of cases observed by him in Vienna
in 1917, although, as he has pointed out, similar epidemics occurred
as early as in 1712. This condition is characterized particularly by
lethargy, facial and oculomotor paralyses and a rise of temperature.
Cases were reported from England and France by various observers
in 1918 and by Pothier, Neal and others in this country in 1919.
It has been suggested frequently that the disease is in some way
associated with influenza. The pathological findings have also been
confused with the African sleeping sickness due to trypanosomes.
After such prodromal symptoms as headache, malaise and drowsiness
with muscular weakness for a few days, a lethargic or stuporous state
usually develops, interrupted occasionally by delirious attacks.
Ptosis has been reported, sometimes with immobility of the pupils.
Paralyses of the facial and eye muscles are very common. Buzzard and
Greenfield[209] after a review of twenty-two cases suggested the
following symptomatological classification:—1. Cases characterized by
hemiplegia, hemianesthesia, hemianopsia, etc.; 2. Cases characterized
by symptoms resembling those of paralysis agitans:—the basal ganglia
group; and 3. Cases characterized by a disturbance of the cranial
nerve functions. In a publication issued recently by the United States
Public Health Service the various types of the disease were summarized
as follows:—1. A clinical affection of the third pair of nerves;
2. Affections of the brain stem and bulb; 3. Affections of the long
tracts; 4. The ataxic type; 5. Affections of the cerebral cortex; 6.
Cases with evidence of spinal cord involvement; and 7. The polyneuritic
type with involvement of the peripheral nerves. The Massachusetts
Department of Public Health has recently recommended the use of the
MacNulty classification, which is quite similar in some respects:—
1. Symptoms of a general nature referable to the central nervous system
with no localizing signs.
2. General symptoms with third nerve paralysis.
3. General symptoms with localizing signs of facial paralysis.
4. General symptoms with localizing signs extending down to the cord.
5. General symptoms with polyneuritic involvements.
6. Mild and abortive cases.
Autopsies have shown meningeal and cortical congestion, degeneration
of the nerve cells, and thickening of the vessels with endothelial
proliferation of the glia. Venous thrombosis and multiple hemorrhages
also occur. In a study of the cerebrospinal fluid Boveri[210] found the
pressure slightly greater in many cases with an increase in the albumen
and globulin content and a mild lymphocytosis in occasional cases. The
findings are not characteristic or of great diagnostic value. Efforts
to isolate the organism responsible for this disease have so far been
unsuccessful.
The mental symptoms associated with encephalitis lethargica have been
studied recently by Abrahamson[211]. He finds that the patient can be
aroused from the initial lethargy and responds quickly and coherently
to questions, relapsing again into an apparent sleep. Some irritability
is shown. The attitude "expresses a desire to be left alone." If the
somnolence disappears it is usually followed by a period of depression.
The patient complains of weariness and inability to sleep. Choreic
manifestations sometimes occur. The somnolence may terminate, on the
other hand, in a profound stupor resembling a drug intoxication with
a restless delirium. Even then the patient can be roused momentarily.
Responses are automatic with no evidences of emotional disturbance.
Flexibilitas cerea is often present. This condition may be followed by
a period of confusion, disorientation and amnesia suggesting Korsakow's
disease. There is usually a period of mental depression with poverty of
thought. Occasional hallucinations were also observed.
An exceedingly important contribution to the literature of encephalitis
lethargica is an analysis recently made of the symptoms shown in
eighteen cases by Kirby and Davis.[212] "The psychic disturbances of
epidemic encephalitis present the general characteristics of an acute
organic type of mental reaction, corresponding more specifically to
a toxic-infectious psychosis. In the acute stages of the disease,
psychic torpor and delirium are the most frequently observed mental
disturbances although other clinical pictures may be encountered,
as the Korsakoff syndrome or more complex mental disorders in which
various affective and trend reactions give a special cast to the
psychotic disturbance." They report two types of sleep disturbance,
hypersomnia and hyposomnia. The former is characterized by drowsiness,
lethargy, stupor or coma, depending entirely on the degree reached. In
the latter the patient is sleepless at night and somnolent during the
daytime. Usually delirium was present at some time in both types of
the disease. Often there was a brief period showing a mild depression
or anxiety, following lethargy or delirium. Euphoria was observed
in a number of instances. In the unrecovered cases they often found
residuals—"depressive affects, emotional elevations, irritability,
explosive reactions, stubbornness, apathy, etc." Their findings may be
summarized perhaps in the statement that "definitely formulated and
persistent trends are infrequent in epidemic encephalitis ... we have
found much evidence of persisting emotional alteration with little
evidence of organic mental defects or dementia."
A review of the statistics of American institutions shows that
psychoses associated with brain and nervous diseases other than
Huntington's chorea and brain tumor, which have already been discussed,
are exceedingly rare. The percentage of cases reported in the New
York hospitals was .95, in the Massachusetts institutions, 1.02, and
in twenty-one other hospitals only 1.56. In a total of 70,987 first
admissions there were only 787 cases (1.1 per cent). The relative
frequency of the various forms is illustrated by the statistics of the
admissions to the New York state hospitals during a period of eight
years. Of 462 cases, 160 were diagnosed as psychoses due to cerebral
embolism; twelve, to meningitis; twenty, to multiple sclerosis;
thirty-eight, to tabes; thirty-four, to acute chorea; and 163, to
other conditions not specified. These figures are astonishing when the
fact that 49,640 patients were admitted during that time is taken into
consideration.