October 23, 2017 E-Pearl of the Week: Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS)

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October 23, 2017

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS)

CANVAS is a presumed autosomal recessive and slowly progressive syndrome characterized by bilateral vestibulopathy, cerebellar ataxia, and generalized sensory neuronopathy (due to marked dorsal root ganglia neuronal loss) with a mean age at onset of 60 years1. When cerebellar atrophy is associated with non-length dependent multi-modality sensory deficits and normal hearing, diagnostic confirmation with video-oculography is appropriate. Impaired visually enhanced vestibulo-ocular reflex is characteristic2. Slowly turning the patient's head while patient visually fixates upon a target will reveal that the compensatory eye movements are saccadic rather than smooth2.

  1. Szmulewicz DJ, Roberts L, McLean CA, MacDougall HG, Halmagyi GM, Storey E. Proposed diagnostic criteria for cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS). Neurol Clin Pract 2016; 6: 61-68.
  2. Szmulewicz DJ, McLean CA, MacDougall HG, Roberts L, Storey E, Halmagyi GM. CANVAS an update: clinical presentation, investigation and management. J Vestib Res 2014; 24: 465-474. 

Submitted by Oana Dumitrascu, MD, Vascular Neurology Fellow - Mayo Clinic Arizona. 

Dr. Dumitrascu reports no disclosures.

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