Spinal cord atrophy in spinocerebellar ataxia type 1.

A 50-year-old man presented with 9-years history of pro gressive ataxia. His father had undiagnosed ataxia. Exa mi nation showed ataxia and pyramidal signs. Brain MRI: olivopontocerebellar atrophy; spine MRI: global spinal cord atrophy (Figure). Genetic test confirmed spinocerebellar ataxia type-1 (SCA1). SCA1 is characterized by cerebellar ataxia with va riable degrees of ophthalmoplegia, pyramidal signs, and peripheral neuropathy1. Spinal cord atrophy was des cribed in other SCA subtypes, but not in SCA12. Prominent pyramidal signs and spinal cord atrophy in SCA1 may be explained by long tracts involvement, as in hereditary spastic paraplegia3. Spinal cord atrophy must be considered in neuroimaging features related to SCA1.