Dystonia in spinocerebellar ataxia type 6.
Spinocerebellar ataxias are heterogeneous disorders with overlapping clinical features. Spinocerebellar ataxia-6 is a dominantly inherited condition characterized by relatively pure ataxia with a paucity of other manifestations including extrapyramidal findings. We report on two patients with genetically proven SCA-6 who had dystonia. One patient presented initially with dystonia, which remained the most disabling problem. Dystonia may occur in SCA-6 and can be disabling.
Sethi
Kapil D
KD
Medical College of Georgia, Augusta, Georgia 30912, USA. Ksethi@neuro.mcg.edu
Jankovic
Joseph
J
D002531:Cerebellum / Q000473:pathology; D004421:Dystonia / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012012:Red Nucleus / Q000473:pathology; D012720:Severity of Illness Index; D020754:Spinocerebellar Ataxias / Q000150:complications*