Hereditary progressive chorea without dementia.
A family with hereditary non-Huntington's chorea is presented. Transmission was autosomal dominant with variable penetrance. Chorea commenced in childhood and affected predominantly the head, face and upper limbs. Dysarthria appeared later, followed in two family members by elements of an axial dystonia. There was no intellectual impairment. Unlike previously described families, symptoms progressed steadily up to the eighth decade, causing considerable physical disability.
Schady
W
W
University Department of Neurology, Manchester Royal Infirmary, UK.
Meara
R J
RJ
D000368:Aged; D002819:Chorea / Q000235:genetics*; D003937:Diagnosis, Differential; D006801:Humans; D006816:Huntington Disease / Q000235:genetics; D008297:Male; D009483:Neuropsychological Tests; D010375:Pedigree