Dystonia as a presenting sign of spinocerebellar ataxia type 1

Abstract
We report on a 39‐year‐old man who presented initially with marked blepharospasm, oromandibular dystonia and retrocollis and one year later developed mild ataxia. Our findings suggest that dystonia can be a disabling presenting sign of SCA1 and support the clinical heterogeneity of SCA1, highlighting the importance of considering this entity in patients combining dystonia and cerebellar ataxia. © 2004 Movement Disorder Society