title: Pure cerebello-olivary degeneration of Marie, Foix, and Alajouanine presenting with progressive cerebellar ataxia, cognitive decline, and chorea.
abstract: 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.
authors.affiliation: Walton Centre for Neurology and Neurosurgery, Liverpool. United Kingdom. shfox@hotmail.com
authors.forename: Susan H
authors.initials: SH
authors.lastname: Fox
authors.forename: Anette
authors.initials: A
authors.lastname: Nieves
authors.forename: Catherine
authors.initials: C
authors.lastname: Bergeron
authors.forename: Anthony E
authors.initials: AE
authors.lastname: Lang
mesh_terms: D001921:Brain / Q000473:pathology*; D002819:Chorea / Q000150:complications / Q000175:diagnosis*; D003072:Cognition Disorders / Q000150:complications / Q000175:diagnosis*; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000473:pathology; D009849:Olivopontocerebellar Atrophies / Q000145:classification* / Q000150:complications / Q000175:diagnosis*; D012720:Severity of Illness Index; D013132:Spinocerebellar Degenerations / Q000150:complications / Q000175:diagnosis*