title: CANVAS an update: clinical presentation, investigation and management.
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.affiliation: Royal Victorian Eye & Ear Hospital, University of Melbourne, Melbourne, Australia.
authors.forename: David J
authors.initials: DJ
authors.lastname: Szmulewicz
authors.affiliation: Department of Anatomical Pathology, Alfred Hospital, Melbourne, Australia.
authors.forename: Catriona A
authors.initials: CA
authors.lastname: McLean
authors.affiliation: Vestibular Research Laboratory, School of Psychology, University of Sydney, Sydney, Australia.
authors.forename: Hamish G
authors.initials: HG
authors.lastname: MacDougall
authors.affiliation: Department of Neuroscience, St Vincent's Hospital, Melbourne, Australia.
authors.forename: Leslie
authors.initials: L
authors.lastname: Roberts
authors.affiliation: Department of Neuroscience, Monash University, Melbourne, Australia.
authors.forename: Elsdon
authors.initials: E
authors.lastname: Storey
authors.affiliation: Department of Neurology, Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: G Michael
authors.initials: GM
authors.lastname: Halmagyi
mesh_terms: D002524:Cerebellar Ataxia / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D003389:Cranial Nerve Diseases / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D006801:Humans; D008607:Intellectual Disability / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D009437:Neuralgia / Q000175:diagnosis / Q000209:etiology / Q000473:pathology; D012027:Reflex, Vestibulo-Ocular / Q000502:physiology; D012189:Retrospective Studies; D020886:Somatosensory Disorders / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D013577:Syndrome; D015837:Vestibular Diseases / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D014724:Vestibular Function Tests
keywords: Cerebellar ataxia; ganglionopathy; neuronopathy; vestibulopathy