Journal article

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

David J Szmulewicz, John A Waterston, Hamish G MacDougall, Stuart Mossman, Andrew M Chancellor, Catriona A McLean, Saumil Merchant, Peter Patrikios, G Michael Halmagyi, Elsdon Storey, J Rucker (ed.), DS Zee (ed.)

Annals of The New York Academy of Sciences | BLACKWELL SCIENCE PUBL | Published : 2011

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). All patients had brain MRI and 22/27 had evidence of cerebellar atrophy involving anterior and dorsal vermis, as well as the hemispheric crus I. Brain and temporal..

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