CHAPTER III

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.