THE PSYCHOSES WITH CEREBRAL ARTERIOSCLEROSIS
Sufficient weight has not been attached heretofore to the important
influence of cerebral arteriosclerosis in the production of mental
diseases. Unquestionably it has been a complicating factor in many of
the generally recognized psychoses which has not been given adequate
consideration. Its relation to involution melancholia as well as
the presenile and senile disorders has been given a great deal of
attention, but cannot as yet be clearly defined. Only in its syphilitic
forms can it be looked upon as contributing to the clinical picture in
general paresis. It is, however, productive of late deterioration in
the chronic alcoholic conditions and in the manic-depressive psychoses
occurring in advanced years. It plays a part frequently in the terminal
stages of dementia praecox. In paranoia and the paranoid conditions of
long standing it often becomes a factor to be reckoned with. Certainly
in the differentiation of the epilepsies of the aged it must be taken
into definite account.
The importance of arteriosclerosis, a term used first by Lobstein some
seventy-five years ago, has long been recognized. Osler in referring to
this subject made the following interesting comment:—"To a majority of
men death comes primarily or secondarily through this portal. The onset
of what may be called physiological arteriosclerosis depends, in the
first place, upon the quality of arterial tissue (vital rubber) which
the individual has inherited and secondarily upon the amount of wear
and tear to which he has subjected it. That the former plays the most
important rôle is shown in the cases in which arteriosclerosis sets in
early in life in individuals in whom none of the recognized etiological
factors can be found. Entire families sometimes show this tendency to
early arteriosclerosis, a tendency which cannot be explained in any
other way than that in the make-up of the machine bad material was used
for the tubing."
Our present knowledge as to the relation of syphilis to this disease
has not changed the significance of the observations made by Osler in
any way. Heredity more than any other one factor undoubtedly determines
the development of both senility and arteriosclerosis. "When," as
Lambert[163] expresses it, "physiological involution anticipates in time
or exceeds in direction, extent and severity normal senescence, the
various senile and arteriosclerotic disorders are the result." It is
as a rule only in the later stages of the disease when focal symptoms
occur or a psychosis develops that hospital care becomes necessary.
Practically any of the vessels of the brain may be involved and it
frequently happens that more than one is affected either directly or
indirectly. The neurological symptoms resulting depend entirely on the
location and extent of the lesion. Lambert[164] has made the following
excellent anatomical classification of the more common arteriosclerotic
processes:—
I. Incipient type.
II. Focal types.
(a) Trunk disorders.
1. Basilar-carotids.
(b) Branch disorders.
1. Inferior cerebellar.
2. Superior cerebellar.
3. Posterior cerebral.
4. Middle cerebral.
5. Anterior cerebral.
(c) Twig disorders.
1. Medullary.
2. Cortical.
Some reference should be made, perhaps, to the focal symptoms resulting
from more or less sharply circumscribed lesions which are productive of
certain fairly well known complexes, whether due to arteriosclerotic
softenings, hemorrhages, or growths. These have been concisely
summarized by Barker[165] somewhat as follows:—
Frontal Lobes—Lesions of the left inferior frontal in righthanded
persons cause motor aphasia. Subcortical involvements cause word
dumbness. Disturbances in the anterior part of the frontal region are
sometimes associated with the Witzelsucht of the German writers—a
tendency towards joking and witticisms.
Central and Paracentral Lobules—Contralateral sensory, motor symptoms
or a combination of the two. Monoplegias, anesthesias and Jacksonian
epilepsies are characteristic. Contralateral tactile agnosia and
apraxia occur, especially in lesions of the left hemisphere. An
involvement of the left side may also cause a homolateral apraxia,
dyspraxia or a tactile agnosia.
Parietal Lobes—Lesions in the anterior part cause contralateral
somesthetic disturbances, tactile agnosia or apraxia. Involvement of
the left angular gyrus may cause optic aphasia or alexia; if deep
enough, hemianopsia results. The voluntary movement of the eye may be
interfered with.
Temporal Lobe—Lesions in the posterior half of the first temporal may
cause Wernicke's sensory aphasia and a subcortical involvement, word
deafness. Bilateral destruction of the first and transverse temporals
causes cortical deafness. Extensive bilateral lesions in the lower
part of these lobes result in mind deafness. Irritative lesions in the
uncinate gyrus lead to hallucinations of taste and smell, with smacking
of the lips and tongue movements.
Island of Reil—Lesions of the anterior part cause symptoms resembling
Broca's motor aphasia. Lesions of the posterior part result in symptoms
suggesting Wernicke's sensory aphasia. Transcortical motor and sensory
aphasia may result.
Occipital Lobes—Lesions of the calcarine area give rise to
hemianopsia, and bicortical involvements lead to cortical blindness.
Bilateral lesions of the lateral surface may cause mind blindness.
Disturbances in the centrum ovale may cause monoplegias or
monoanesthesias, and lesions in the corpus callosum, apraxic symptoms.
Characteristic of cerebellar lesions are ataxias and disturbances of
equilibrium, often with vertigo and paroxysmal vomiting.
An involvement of the corpora quadrigemina may cause pupillary changes,
unilateral or bilateral paralysis of eye muscles, nystagmus, visual
disturbances, deafness and ataxia or anesthesia.
Lesions of the cerebral peduncles may give rise to very characteristic
syndromes. If the tegmentum and pes pedunculi (basis pedunculi) are
both involved, there may be a complete hemiplegia of the opposite side
with an oculomotor paralysis on the same side (Weber-Gubler syndrome).
Or there may be in addition to this a marked tremor in the limbs of the
paralyzed side (Benedikt's syndrome). A unilateral oculomotor paralysis
may be combined with a cerebellar ataxia (Nothnagel's syndrome).
The thalmic syndrome of Déjerine and Roussy shows a contralateral
hemianesthesia, violent and persistent pains on the anesthetic side,
hemiataxia, hemichorea or hemiathetosis, slight temporary hemiparesis
and sometimes hyperesthesia. Lesions further back, possibly involving
the internal capsule, may cause hemianesthesia of touch, pain and
temperature senses.
S. A. K. Wilson in 1912 called attention to a particularly important
syndrome, designated by him as "progressive lenticular degeneration"
and characterized by dysarthria, dysphagia, general tremors of the
extremities, forced laughing and crying, muscular rigidities and
contractures, with a slight intellectual impairment. Interesting
features of this disease complex are that it is familial in type, but
not hereditary, comes on early in life, usually progressing to a fatal
termination, and is associated with a cirrhosis of the liver which
is not alcoholic in origin. At autopsy degenerations of the nucleus
lentiformis have been found. J. Ramsey Hunt in 1916 called attention to
the association of both paralysis agitans and Huntington's chorea with
lesions in the globus pallidus. Oppenheim has recently differentiated
a striatum syndrome to which he gave the name "dystonia musculorum."
Difficulties in writing, tremors, disturbance of the gait, rigidities,
tonic and clonic movements of the muscles and other neurological
symptoms are present. Several cases reported by Abrahamson in 1920
showed definite emotional disturbances. Cecile and Oskar Vogt have
recently (1919) studied the striatum lesions from a standpoint of both
pathology and symptomatology. As summarized by Lhermitte[166] their work
shows that athetosis, paralysis agitans, Huntington's chorea, dystonia
musculorum, probably paralysis agitans and various other neurological
syndromes are to be attributed directly to conditions involving the
striate bodies. Prominent among these are softenings and hemorrhages
which may result from arteriosclerosis. In view of these facts a
careful study of the focal lesions associated with the arteriosclerotic
disorders is exceedingly important.
The pathological processes involved have been carefully studied by
Heubner and others. He was originally of the opinion that cerebral
arteriosclerosis was always of specific origin. Baumgarten, however,
subsequently showed that this was not the case. The more characteristic
changes in the larger vessels manifest themselves in the form of
patches of atheromatous thickening so common at autopsy. As a result
of degenerative changes in the elastica and media, and a consequent
weakening of the vessel wall, intimal thickening takes place. This is
not the circular, uniform, concentric involvement found in syphilitic
processes but a localized proliferation of the intima at some one
point. There may be an infiltration of colloid and calcareous material
in the media. This leads to further intimal thickening. In the smaller
vessels arteriocapillary fibrosis has been described—a uniform
thickening of the vessel walls with a connective tissue formation.
Endarteritis obliterans, first described by Friedländer in 1876, is
probably always of syphilitic origin.
In addition to the vascular changes in the cerebral vessels
Kraepelin[167] finds usually atheromatous changes in the aorta and its
branches, particularly the coronaries, with ulcerations or calcareous
plates, hypertrophy and dilatation of the heart, myocarditis,
interstitial nephritis and infarctions of various organs. At autopsy
the dura and pia are usually thickened and adherent, with a general
atrophy of the cerebral convolutions. There are often fresh
hemorrhages under the membranes as well as cyst formations and
dilatation of the brain ventricles. He particularly emphasizes a
splitting of the elastica in the larger cerebral vessels with a
thickening and tortuosity, fatty infiltration and calcareous deposits.
Hyaline degeneration is common in the elastica and muscularis with
fatty granular cells in the adventitia. Capillary aneurysms are often
found. Glia proliferation is to be expected in the surrounding area. A
condition described by Alzheimer as perivascular gliosis often occurs.
There is a disappearance of the perivascular nervous elements with
consequent proliferation of the neuroglia. In a general way Kraepelin
differentiates several distinct pathological groups—a diffuse cortical
involvement, circumscribed processes in the neighborhood of vessels,
hemorrhages and softenings. There is also a loss of nerve fibres
which are replaced by neuroglia. Binswanger has described a "chronic
subcortical encephalitis" due to arteriosclerosis. This consists of an
atrophy of the white matter due to an involvement of the deeper marrow
vessels. Large gaps and lacunae are found in the course of the vessels.
There is an extensive atrophy of the fibres and there may be occasional
foci of softening. As a general rule involvement of the large vessels
is liable to affect the medullary substance while sclerosis of the
smaller vessels leads to cortical disturbances. It is also possible
to have extensive lesions without mental symptoms and well developed
psychoses with only a slight physical basis. The site of the damage to
the vessels determines this. On the other hand, the mental condition
may be due to cardiovascular complications resulting usually in
anxiety psychoses. The symptomatology may be complicated by senility,
alcoholism or syphilis.
Clinically Kraepelin[168] divides the arteriosclerotic psychoses into
deteriorations, or milder forms of mental enfeeblement, dementias,
depressions, excitements, late epilepsies, and apoplectic dementia.
In the milder forms there is a gradual change in the entire psychic
personality, with a later development of more marked changes, either
physical, mental or both. The early symptoms are a general reduction
of the mental capacity and an impairment of memory. The patient tires
easily and loses all evidences of energy, with no inclination to
undertake anything new. Familiar names and dates are forgotten. Recent
occurrences are particularly lost to memory. The real is confused with
the false. In business the patient becomes careless and unreliable,
overlooks important transactions and forgets appointments. There are
often subjective feelings of impending illness. The mood becomes
depressed, whining and tearful. Irritability and outbursts of anger
occasionally appear, characterized by a marked emotional instability,
varying rapidly from tears to laughter. Suicidal tendencies are
sometimes noted. Mild confusional states may be induced by alcoholic
indulgences. Early physical symptoms are headache, sensations of
fulness and pressure in the head, followed by a feeling of dizziness,
fatigue, exhaustion, debility, etc. Sooner or later, following a
seizure of some kind, neurological signs appear—drooping of the
mouth, lateral deviation of the tongue, weakness of an arm, dragging
of one leg, loss of sensation on one side, ankle clonus, an increase,
decrease or inequality of the patellar reflexes, and sometimes a
Babinski reflex. The pupils are very likely to be unequal and sluggish
in reaction. The features present a tired, sleepy expression and
speech becomes tremulous and monotonous. There may be a difficulty
in finding words, or the misuse of words. There are usually tremors
of the fingers and movements are uncertain, the gait being unsteady.
Romberg's symptom may be present. Dizzy spells and fainting attacks
also occur, sometimes followed by genuine convulsions. Apoplectiform
seizures may be observed, with unconsciousness for hours or days. These
may be followed by sensory or motor aphasia, unilateral paralysis with
or without disturbances of sensation, hemianopsia, alexia, agraphia,
asymbolism or apraxia. Cardiac disturbances with anxieties are often
complications. These apoplectiform and other severe attacks sometimes
occur a long time after mental symptoms have appeared. They are likely
to recur, mental deterioration progressing rapidly with the repetition
of the seizures.
Apprehension is much disturbed and memory weakened, in the advanced
cases of deterioration. The patients cannot remember anything for
more than a short time. They become disoriented as to time, place and
person and forget their own names. Genuine "confabulation" sometimes
appears. There are often confusional and delirious states. The mood is
frequently depressed or anxious, sometimes irritable or quarrelsome
and at times humorous. There is a group of cases showing genuine
depressions, usually with hypochondriacal delusions, sometimes with
delusions of persecution, self-accusation, and ideas of sinfulness.
Even delusions of grandeur are observed. Hallucinations are not
infrequent in these cases. States of excitement may intervene with
occasional delusions and confused attacks. These excitements are
usually of the agitated, restless type, sometimes with suicidal
inclinations. Stuporous or even cataleptic states may follow. In the
highest forms of excitement sensory or motor aphasia may develop,
often with speech disturbances, sometimes of a genuine scanning
type. Paraphasias are common. The writing is ataxic or paragraphic.
Ideational or motor apraxia often is a symptom. Cyanoses and other
evidences of general arteriosclerotic involvement appear. There may be
an albuminuric retinitis. Albumen and sometimes sugar appear in the
urine. The radials and temporals are thickened or hardened and cardiac
murmurs are often found. Blood pressure is greatly increased in many
instances, although Romberg found it in only ten per cent of his cases.
Sleep is usually interfered with to a marked degree.
In a certain number of the more advanced cases of arteriosclerosis late
epilepsies appear. The attacks usually begin between the forty-fifth
and sixty-fifth years. There may be fainting spells or genuine
convulsions recurring at frequent intervals. These may be associated
with brief periods of delirium or may even occur without loss of
consciousness. Forgetfulness and mental enfeeblement soon appear in
such cases. They also show physical changes with tremors, disturbed
reflexes, paralyses, increased blood pressure, etc. Alcoholism seems
to be a strongly predisposing factor in this form of arteriosclerotic
disorder. Kraepelin found that the epileptic attacks almost invariably
appeared in cases which showed a previous history of alcoholic excesses.
In nearly half of his cases Kraepelin found apoplectiform attacks
appearing without any marked psychosis preceding them. In some
instances no mental symptoms appeared for many years. The attacks
were, however, immediately followed, usually, by periods of confusion
and clouding, sometimes of excitement and violence. The acute
disturbance as a rule subsides rather quickly and clears up partially
or completely. Usually there remains a memory defect, an increased
fatigability and a depressed or irritable mood. These he refers to as
cases of apoplectic deterioration or mental enfeeblement. Recurrent
apoplectiform seizures may result in excitement, depressions or
deliria. Gradual progressive deterioration is the usual picture. As a
general rule the cases with marked excitements, depressions and deliria
are of short duration and have a bad prognosis.
Kraepelin finds that the arteriosclerotic psychoses appear a decade
earlier than the senile psychoses. Less than one per cent developed at
the age of forty; 2.7 per cent at forty-five; 3.7 per cent at fifty;
7.4 per cent at fifty-five; twenty-two per cent at sixty; twenty-two
per cent at sixty-five; 18.57 per cent at seventy; twelve per cent at
seventy-five, etc. In the cases observed at a particularly early age
he believes heredity to be a very important factor. Seventy-one and
five-tenths per cent of his cases were men. Sixty-two per cent of the
men and fifty-three per cent of the women were less than sixty-five
years of age. The epileptic and demented forms appear earlier than the
apoplectiform variety. Arteriosclerotic involvement of the smaller
vessels occurs earlier than that of the larger arteries. Kraepelin
found alcoholism more common in the history of his cases than syphilis.
He is uncertain whether specific infections can produce a genuine
arteriosclerosis or not.
Erb has shown that by the experimental injection of adrenalin into
the blood stream artificial arteriosclerosis can be produced, with
an increase of blood pressure, splitting of the elastica, thickening
of the vessel walls and aneurysm formation. Thoma considers alcohol,
tobacco, coffee, tea, and infectious poisons important causes. Cramer
found the disease more common in innkeepers, actors, directors,
officers, bankers and parliamentarians. Alcohol, syphilis, overwork
and high living are important etiological factors. Kraepelin assumes
the existence of certain metabolic products in the blood, possibly the
result of infections which affect blood pressure and the structure of
the vessel walls during a period of lowered resistance.
The pathological changes associated with the arteriosclerotic psychoses
are quite clearly demarcated. Clinical differentiations, however, are
not so well established. There is some question as to the justification
of the separate entities into which Kraepelin would divide the
arteriosclerotic processes. For statistical purposes the Association's
committee felt that a determination of the frequency of occurrence of
the arteriosclerotic group as a whole is all that should be attempted
at this time. The following suggestions were offered in the manual as
to the delimitations of these conditions:—
"The clinical symptoms, both mental and physical, are varied depending
in the first place on the distribution and severity of the vascular
cerebral disease and probably to some extent on the mental make-up of
the person.
"Cerebral physical symptoms, headaches, dizziness, fainting attacks,
etc., are nearly always present, and usually signs of focal brain
disease appear sooner or later (aphasia, paralysis, etc.).
"The most important mental symptoms (particularly if the
arteriosclerotic disease is diffuse) are impairment of mental tension,
_i.e._, interference with the capacity to think quickly and accurately,
to concentrate and to fix the attention; fatigability and lack of
emotional control (alternate weeping and laughing), often a tendency
to irritability is marked; the retention is impaired and with it there
is more or less general defect of memory, especially in the advanced
stages of the disease, or after some large destructive lesion occurs.
"Pronounced psychotic symptoms may appear in the form of depression
(often of the anxious type), suspicions or paranoid ideas, or episodes
of marked confusion.
"To be included in this group are the psychoses following cerebral
softening or hemorrhage, if due to arterial disease. (Autopsies in
state hospitals show that in arteriosclerotic cases softening is
relatively much more frequent than hemorrhage.)
"Differentiation from senile psychosis is sometimes difficult
particularly if the arteriosclerotic disease manifests itself in
the senile period. The two conditions may be associated; when this
happens preference should be given in the statistical report to the
arteriosclerotic disorder.
"High blood pressure, although usually present, is not essential for
the diagnosis of cerebral arteriosclerosis."
In the 49,640 admissions to the New York state hospitals during a
period of eight years the 2,318 cases diagnosed as psychoses with
arteriosclerosis constituted 4.67 per cent of the total number. In
twenty-one hospitals in other states there were 18,336 admissions, of
which 492, or 2.68 per cent, were cases of arteriosclerosis. On the
other hand, the Massachusetts hospitals show 9.63 per cent of their
first admissions during 1919 as arteriosclerotic psychoses. There would
appear to be no way to harmonize these dissimilar findings unless it
is merely a question of differentiation between the senile psychoses
and those due to arteriosclerosis. In a total of 70,987 admissions to
all institutions, there were 3,100 cases of arteriosclerotic psychoses,
a percentage of 4.36. It is worthy of note that in all of the various
groups of institutions the percentage of senile and arteriosclerotic
cases combined is practically the same. This would strongly suggest
varying standards of diagnosis which will undoubtedly be reconciled in
time. It is only recently that any great amount of attention has been
given to the psychoses due to arteriosclerosis and it must be confessed
that there has been entirely too great a tendency to dismiss without
further interest as senile psychoses all mental disturbances occurring
in persons of advanced years. On the other hand, the custom of basing
a diagnosis of arteriosclerotic psychosis on the mere presence of an
increased blood pressure without the existence of any of the other
symptoms which characterize that condition indicates, if nothing else,
the necessity of a greater uniformity in our methods of diagnostic
procedure.