Huntington's chorea. Report of 3 cases and review of the literature.

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Abstract
Three cases of Huntington''s chorea with onset before age 10 yr are reported. Each child presented with rigidity and indistinct speech, and there was progressive deterioration. Necropsy examination confirmed the diagnosis in 2. A review of reports showed a further 43 cases with onset before 10 yr. The rigid variety of disease was seen most often, but isolated chorea and isolated progressive mental deterioration occurred. Fits were common but occurred late and were often difficult to control. Dysarthria was common and occurred early. The duration of illness was variable and ranged from 2-38 yr. Symptoms can occur in a child before appearing in the affected parent (most likely the father). Affected siblings develop the disease early, often in the 1st decade. Siblings of patients with onset before age 10 yr who are unaffected by age 25 yr had only an 8% chance of developing the disease, compared with a 50% chance in unselected individuals of the same age.

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