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."