CANVAS syndrome: management

Vestibular tests

• Visually enhanced vestibulo-ocular reflex (VVOR): doll's head reflex
• Vestibulo-ocular reflex (VOR)
• Optokinetic reflex (OKR)
• Smooth pursuits (SP)
• Caloric response

Nerve conduction studies (NCS): features

Magnetic resonance imaging (MRI): features

Nerve ultrasound

Acronyms

References

1. Migliaccio AA, Halmagyi GM, McGarvie LA, Cremer PD. Cerebellar ataxia with bilateral vestibulopathy: description of a syndrome and its characteristic clinical sign. Brain 2004; 127:280-293.
2. Szmulewicz DJ, Seiderer L, Halmagyi GM, Storey E, Roberts L. Neurophysiological evidence for generalized sensory neuronopathy in cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome. Muscle Nerve 2015; 51:600-603.
3. Szmulewicz DJ, Waterston JA, Halmagyi GM, et al. Sensory neuropathy as part of the cerebellar ataxia neuropathy vestibular areflexia syndrome. Neurology 2011; 76:1903-1910.
4. Lobo CC, Wertheimer GSO, Schmitt GS, et al. Cranial nerve thinning distinguishes RFC1-related disorder from other late-onset ataxias. Mov Disord Clin Pract 2024; 11:45-52.
5. Pelosi L, Mulroy E, Leadbetter R, et al. Peripheral nerves are pathologically small in cerebellar ataxia neuropathy vestibular areflexia syndrome: a controlled ultrasound study. Eur J Neurol 2018; 25:659-665.
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Related checklists

• CANVAS syndrome: clinical features