Pure cerebello-olivary degeneration of Marie, Foix, and Alajouanine presenting with progressive cerebellar ataxia, cognitive decline, and chorea.

Parenchymatous cerebellar cortical atrophy (CCA) usually presents with a "pure" cerebellar ataxia. We describe a patient with a sporadic, late-onset progressive cerebellar ataxia plus cognitive decline and chorea who had CCA at post mortem. We discuss this unique case in the current context of classification of idiopathic cerebellar ataxia. Copyright 2003 Movement Disorder Society