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.

Keywords: Cerebellar ataxia; ganglionopathy; neuronopathy; vestibulopathy.