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