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Charts showing incidence of Huntington's Chorea in the families and descendants of George M
and his sister Mary M
. L, presented to American Medico-Psychological
Association, June 2, 1909

(Names printed in bold font type represent patients who have suffered from Huntington's Chorea)

DESCENDANTS OF GEORGE M

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HEREDITARY CHOREA (HUNTINGTON'S).

BY BEATRICE A. STEVENSON, M. D., AND H. D. PURDUM, M. D., Assistant Physicians, Northern Michigan Asylum, Traverse City, Michigan.

This case has been selected, from a comparatively large number admitted to this institution, as one to satisfactorily demonstrate practically all of the cardinal features of this most interesting and obscure disease, Huntington's chorea.

A. W., female, was admitted to the Northern Michigan Asylum, January 15, 1903, and died May 26, 1908.

The abstract of the history is as follows: Father and mother were natives of England, were not related and were, respectively, 23 and 21 years of age at the time of the patient's birth. No nervous peculiarities were recorded in reference to the paternal side of the family.

The maternal records revealed the following: Maternal grandfather, two aunts, three uncles, ten cousins, mother and two sisters had, or now have, this disease. Two brothers have been sentenced for arson and theft and a son of the patient is now in a reformatory.

The patient was born in the State of Michigan, in 1860. The developmental period was uneventful. During her early adult life she enjoyed fair physical health, though she was considered to be of a nervous temperament and was subject to attacks of fainting, palpitation and vasomotor irritability. She married at the age of 24 and had seven children. Her married life was an unhappy one and was attended by hard work and mental stress.

The first choreiform movements were noted at the age of 36. These were soon followed by gradual progressive mental deterioration with heightened emotional feelings. Nine years after the first choreic movements were observed the patient became depressed, entertained fears of becoming insane, had delusions of persecution and grandeur, together with auditory and visual hallucinations. Later on she became irritable, quarrelsome and aggressive, and demonstrated complete loss of self-control by violent outbursts of passion.

On being admitted to the hospital, she was emaciated, weak and in a much neglected condition. Her lips and teeth were covered with sordes, tongue was coated and breath offensive. Temperature was 99.6; pulse, 110. Respirations could not be satisfactorily counted because of the more or less constant choreic spasms which involved the face, trunk and extremities. Patient was unable to stand, walk or sit in a chair without support on account of her generally weakened state and incessant spasmodic movements. A thorough physical examination of the internal organs failed to reveal any evidence of disease. Deep reflexes were mark

edly exaggerated; superficial, slightly so. Pupils were equal, circular, and reacted to light and accommodation. The special senses were active as far as could be determined. Urine contained a trace of albumin and a few hyalin and granular casts. Blood was light red in color and its coagulability lessened. Hemoglobin was 68 per cent; erythrocytes, 3,468,000; leucocytes, 4948. A differential count did not show any departure from the normal proportion of the various white cells.

One week after admission a mental status was made and the following recorded:

Patient is cared for in bed and requires a great deal of special attention on account of her untidy habits and destructive tendencies. Appetite is good, but sleep insufficient. She is incapable of caring for herself and has been noisy, irritable and restless. Movements are extensive, involving the entire body. The facial expression is constantly changing. The extremities jerk and the body is thrown from one side of the bed to the other. She picks at and destroys the bed clothing and makes a clucking noise with her lips and tongue. A sustained conversation is impossible. Her replies to questions are irrelevant and with great difficulty understood, as she cannot articulate distinctly. Occasionally she whispers to herself or screams as if hallucinated. Deterioration is marked, consciousness clouded and orientation imperfect.

Memory for both remote and recent events is poor, and chronological sequence markedly impaired.

Ideation is imperfect, patient being unable to associate properly. Some distractibility with flight of ideas and looseness of thought connection is noted.

Judgment is greatly affected, this being partially due to memory impairment and partially to delusional ideas. The delusions entertained are of an expansive and depressive nature, and are evidenced by her actions and fragmentary remarks. She has no insight into her condition.

The emotional state is one of more or less constant irritability. The affect is completely changed and patient is violent toward those who care for her. The finer sensibilities are blunted. Hunger, fatigue and sexual feelings are more or less in abeyance.

Will power is diminished, and impulsive and compulsive acts occur, patient having attempted suicide several times since being admitted. Occasionally she resents attention and becomes quite aggressive and violent.

The above physical condition and mental peculiarities remained more or less stationary except that the mind gradually deteriorated and the body became weaker. Suicidal tendencies and delusional ideas faded as the dementia progressed. It was difficult to prevent bed sores, as the friction produced by the constant muscular spasms wore away the skin. It became necessary to remove the three central incisors from the upper jaw, as the continuous forcible movements of the lower jaw had loosened them until they stood out between the lips. In the early part of April, 1908, patient contracted acute pulmonary tuberculosis and death occurred May 26, 1908. Autopsy, 15 hours after death.

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