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CANVAS an update: clinical presentation, investigation and management.
J Vestib Res. 2014; 24(5-6):465-74.JV

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.

Authors+Show Affiliations

Royal Victorian Eye & Ear Hospital, University of Melbourne, Melbourne, Australia.Department of Anatomical Pathology, Alfred Hospital, Melbourne, Australia.Vestibular Research Laboratory, School of Psychology, University of Sydney, Sydney, Australia.Department of Neuroscience, St Vincent's Hospital, Melbourne, Australia.Department of Neuroscience, Monash University, Melbourne, Australia.Department of Neurology, Royal Prince Alfred Hospital, Sydney, Australia.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

25564090

Citation

Szmulewicz, David J., et al. "CANVAS an Update: Clinical Presentation, Investigation and Management." Journal of Vestibular Research : Equilibrium & Orientation, vol. 24, no. 5-6, 2014, pp. 465-74.
Szmulewicz DJ, McLean CA, MacDougall HG, et al. CANVAS an update: clinical presentation, investigation and management. J Vestib Res. 2014;24(5-6):465-74.
Szmulewicz, D. J., McLean, C. A., MacDougall, H. G., Roberts, L., Storey, E., & Halmagyi, G. M. (2014). CANVAS an update: clinical presentation, investigation and management. Journal of Vestibular Research : Equilibrium & Orientation, 24(5-6), 465-74. https://doi.org/10.3233/VES-140536
Szmulewicz DJ, et al. CANVAS an Update: Clinical Presentation, Investigation and Management. J Vestib Res. 2014;24(5-6):465-74. PubMed PMID: 25564090.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - CANVAS an update: clinical presentation, investigation and management. AU - Szmulewicz,David J, AU - McLean,Catriona A, AU - MacDougall,Hamish G, AU - Roberts,Leslie, AU - Storey,Elsdon, AU - Halmagyi,G Michael, PY - 2015/1/8/entrez PY - 2015/1/8/pubmed PY - 2015/12/31/medline KW - Cerebellar ataxia KW - ganglionopathy KW - neuronopathy KW - vestibulopathy SP - 465 EP - 74 JF - Journal of vestibular research : equilibrium & orientation JO - J Vestib Res VL - 24 IS - 5-6 N2 - 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. SN - 1878-6464 UR - https://wwww.unboundmedicine.com/medline/citation/25564090/CANVAS_an_update:_clinical_presentation_investigation_and_management_ DB - PRIME DP - Unbound Medicine ER -