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著者: David J Szmulewicz, Catriona A McLean, Hamish G MacDougall, Leslie Roberts, Elsdon Storey, G Michael Halmagyi
雑誌名: J Vestib Res. 2014;24(5-6):465-74. doi: 10.3233/VES-140536.
Abstract/Text
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.
PMID 25564090 J Vestib Res. 2014;24(5-6):465-74. doi: 10.3233/VES-140536.
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