CANVAS an update: clinical presentation, investigation and management

David Joshua Szmulewicz, Catriona Ann McLean, Hamish G MacDougall, Leslie Roberts, Elsdon Storey, Michael Halmagyi

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BACKGROUND: Cerebellar Ataxia with Neuropathy and bilateral Vestibular Areflexia Syndrome (CANVAS) is a multi-system ataxia which results in cerebellar ataxia, a bilateral vestibulopathy and a somatosensory deficit. This sensory deficit has recently been shown to be a neuronopathy, with marked dorsal root ganglia neuronal loss. The characteristic oculomotor clinical sign is an abnormal visually enhanced vestibulo-ocular reflex. OBJECTIVE: To outline the expanding understanding of the pathology in this condition, as well as diagnostic and management issues encountered in clinical practice. METHODS: Retrospective data on 80 CANVAS patients is reviewed. RESULTS: In addition to the triad of cerebellar impairment, bilateral vestibulopathy and a somatosensory deficit, CANVAS patients may also present with orthostatic hypotension, a chronic cough and neuropathic pain. Management of falls risk and dysphagia is a major clinical priority. CONCLUSIONS: CANVAS is an increasingly recognised cause of late-onset ataxia and disequilibrium, and is likely to be a recessive disorder.
Original languageEnglish
Pages (from-to)465 - 474
Number of pages10
JournalJournal of Vestibular Research: Equilibrium and Orientation
Issue number5-6
Publication statusPublished - 2014

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