Craniocervical Segmental Dystonia in the Spinocerebellar Ataxia Type 2

ABSTRACT

The spinocerebellar ataxia type 2 (SCA 2) is an autosomal dominant cerebellar ataxia that commonly presents with cerebellar ataxia, hyporeflexia, and slow saccades. Recent clinical series described movement disorder in the SCA 2 such as Parkinsonism or dystonia. Dystonia can be observed in and even be the presenting feature of the SCA 2. We report two patients with genetically confirmed SCA 2 displaying a slowly progressive syndrome combined with cerebellar ataxia and craniocervical segmental dystonia.