[Case of benign familial chorea].

Abstract

The authors present a family affected by benign chorea. It is a hereditary disease, characterized by dominant autosomic transmission, with incomplete penetrance. Genetic, clinical and evolutional features are reported. The choreic movements rise in the first twenty years of life but psychological deterioration, epilepsy or other neurological symptoms did not emerge. Pharmacological treatments did not help in such non progressive abnormal movements in our subjects. Computerized tomographic scan resulted negative in four subjects, examined in different phases of the disease. This confirms the hypothesis of a functional, rather than structural, lesion in this benign form of familial chorea.