Benign hereditary chorea Clinical and genetic aspects

Abstract

Five patients from two families in Wales are described who have a benign disorder characterized by lifelong, non‐progressive choreic movements, but without mental deterioration or other features of Huntington's disease. The clinical picture and natural history of this specific disorder are discussed, with particular emphasis on its distinction from Huntington's disease. Genetic data from previously reported kindreds are reviewed, and it is concluded that the disorder follows autosomal dominant inheritance, with reduced penetrance in the female. The importance is stressed of recognizing this disorder and not confusing it with Huntington's disease, if serious errors in prognosis and genetic counselling are to be avoided.