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

Authors

  • Susan H. Fox MRCP, PhD,

    Corresponding author
    1. Walton Centre for Neurology and Neurosurgery, Liverpool. United Kingdom
    • Walton Centre for Neurology and Neurosurgery, Lower Lane, Fazakerley, Liverpool L9 7LJ, United Kingdom
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  • Anette Nieves MD,

    1. Movement Disorders Centre, Toronto Western Hospital and Center for Research in Neurodegenerative Disease, Toronto, Canada
    Current affiliation:
    1. Department of Neurology, Robert Wood Johnson Medical School, Camden, NJ
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  • Catherine Bergeron MD, FRCPC,

    1. Division of Neuropathology, Toronto Western Hospital and Center for Research in Neurodegenerative Disease, Toronto, Canada
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  • Anthony E. Lang MD, FRCPC

    1. Movement Disorders Centre, Toronto Western Hospital and Center for Research in Neurodegenerative Disease, Toronto, Canada
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  • A videotape accompanies this article.

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. © 2003 Movement Disorder Society

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