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Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS)

Case contributed by Grace Carpenter
Diagnosis almost certain

Presentation

Recent increase in unsteadiness with associated sensory peripheral neuropathy.

Patient Data

Age: 75 years
Gender: Female

BRAIN: Cerebellar atrophy is marked, especially in the region of the lower vermis. Moderate generalized cerebral atrophy. Brainstem is normal. Moderate midbrain atrophy. Diffuse white matter hyperintensity consistent with small vessel type ischemia.

CERVICAL SPINE: Spinal canal is capacious. Slight loss of the
normal cervical enlargement. Spinal cord caliber appears below average.

Case Discussion

This case demonstrates a patient with cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS). It depicts common MRI findings of focal cerebellar atrophy, particularly in the vermis, as well as spinal cord atrophy, most notably in the cervical region. This patient was diagnosed with CANVAS 10 years prior to this presentation at a tertiary center and was found to have a positive family history of neurodegenerative disorders; this patient has two family members who suffer from multiple sclerosis.

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