Cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS): a review of the clinical features and video-oculographic diagnosis

David Szmulewicz, John Waterston, Hamish MacDougall, Stuart Mossman, Andrew Chancellor, Catriona Ann McLean, Saumil Merchant, Peter Patrikios, Michael Halmagyi, Elsdon Storey

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Abstract

The association of bilateral vestibulopathy with cerebellar ataxia was first reported in 1991 and delineated as a distinct syndrome with a characteristic and measurable clinical sign-an absent visually enhanced vestibulo-ocular reflex-in 2004. We reviewed 27 patients with this syndrome and show that a non-length-dependent sensory deficit with absent sensory nerve action potentials is an integral component of this syndrome, which we now call cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS).
Original languageEnglish
Pages (from-to)139 - 147
Number of pages9
JournalAnnals of the New York Academy of Sciences
Volume1233
DOIs
Publication statusPublished - 2011

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