Uncovering Neuropathic Pain in CANVAS: Lessons from a Case-Based Literature Review

Cerebellar ataxia with neuronopathy and vestibular areflexia syndrome (CANVAS) is a rare, late-onset neurodegenerative disorder caused by biallelic intronic AAGGG repeat expansions in the RFC1 gene. Its phenotype is heterogeneous, ranging from isolated sensory neuronopathy to the full clinical triad....

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Audiologists and vestibular specialists encountering CANVAS patients should be alert to co-occurring neuropathic pain, as it may be underdiagnosed; however, this case-based review does not provide sufficient evidence to change pain management protocols.

CANVAS is a rare but important differential diagnosis for vestibular areflexia, and recognising its full symptom burden — including neuropathic pain — is critical for appropriate multidisciplinary referral.

1. 01CANVAS (cerebellar ataxia with neuronopathy and vestibular areflexia syndrome) is a rare, progressive neurodegenerative disorder.
2. 02Vestibular areflexia (complete loss of balance organ function) is a hallmark feature assessed by audiologists and vestibular specialists.
3. 03Neuropathic pain (nerve pain) is highlighted as an underrecognised symptom in CANVAS.
4. 04Case-based literature review format draws on real patient cases and published literature rather than a controlled study.
5. 05Published in Pain and Therapy (Springer), targeting a pain medicine audience with relevance to vestibular clinicians.

PMID

42525371

DOI

10.1007/s40122-026-00867-2.

Journal

Pain and Therapy

Publication type

review

Evidence level

5

Population

Patients with CANVAS (cerebellar ataxia with neuronopathy and vestibular areflexia syndrome)

Intervention

Characterisation of neuropathic pain in CANVAS syndrome