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CANVAS an update: Clinical presentation, investigation and management

Issue title: The Vestibular System: A Clinical and Scientific Update in Siena. In honor of Professor Daniele Nuti, Siena, Italy, April 5–6, 2013

Guest editors: Marco Mandalàx, Stefano Ramaty and David S. Zee

Article type: Research Article

Authors: Szmulewicz, David J.a; * | McLean, Catriona A.b | MacDougall, Hamish G.c | Roberts, Leslied | Storey, Elsdone | Halmagyi, G. Michaelf

Affiliations: [a] Royal Victorian Eye & Ear Hospital, University of Melbourne, Melbourne, Australia | [b] Department of Anatomical Pathology, Alfred Hospital, Melbourne, Australia | [c] Vestibular Research Laboratory, School of Psychology, University of Sydney, Sydney, Australia | [d] Department of Neuroscience, St Vincent's Hospital, Melbourne, Australia | [e] Department of Neuroscience, Monash University, Melbourne, Australia | [f] Department of Neurology, Royal Prince Alfred Hospital, Sydney, Australia | [x] Otological and Skull Base Surgery Department, S.M. delle Scotte Hospital, Siena, Italy | [y] Department of Electrical, Computer and Biomedical Engineering, University of Pavia, Pavia, Italy

Correspondence: [*] Corresponding author: David Szmulewicz, Balance Disorders & Ataxia Service, Royal Victorian Eye and Ear Hospital, 32 Gisborne Street, East Melbourne VIC, 3002, Australia. Tel.: +613 9929 8666; Fax: +613 9012 4465; E-mail: [email protected]

Keywords: Cerebellar ataxia, vestibulopathy, neuronopathy, ganglionopathy

DOI: 10.3233/VES-140536

Journal: Journal of Vestibular Research, vol. 24, no. 5-6, pp. 465-474, 2014

Received 14 May 2014
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Accepted 14 August 2014
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Published: 17 December 2014
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Abstract

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.

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