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.
Fox
Susan H
SH
Walton Centre for Neurology and Neurosurgery, Liverpool. United Kingdom. shfox@hotmail.com
Nieves
Anette
A
Bergeron
Catherine
C
Lang
Anthony E
AE
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*