Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS)

Auris, Nasus, Larynx
Masakatsu TakiShigeru Hirano

Abstract

Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS) is a novel ataxic disorder consisting of the triad of cerebellar impairment, bilateral vestibular hypofunction, and a somatosensory deficit. We report the first Japanese case of CANVAS. The patient is a 68-year-old Japanese male. He was referred to our university for further evaluation of progressive gait disturbance and ataxia. He exhibited horizontal gaze-evoked nystagmus and sensory deficit. Nerve conduction studies showed sensory neuronopathy. Magnetic resonance imaging showed the atrophy of vermis but not of the brainstem. The caloric stimulation and video head impulse test (vHIT) showed bilateral vestibulopathy. The visually enhanced vestibulo-ocular reflex (VVOR) was also impaired. In addition to neurological and electrophysiological examinations, simple neuro-otological examinations (i.e., caloric stimulation, vHIT, and VVOR) may reveal more non-Caucasian cases.

Citations

Feb 12, 2019·Journal of Neuroimmune Pharmacology : the Official Journal of the Society on NeuroImmune Pharmacology·Natalie M ZahrEdith V Sullivan
Feb 11, 2020·Brain : a Journal of Neurology·Andrea CorteseMary M Reilly
Jan 5, 2019·Rinshō shinkeigaku = Clinical neurology·Kyoko MarutaYoshito Sonoda
Jul 23, 2020·Journal of Human Genetics·Mai TsuchiyaYoshihisa Takiyama
Sep 11, 2020·Journal of Neurology·Natalia DominikHenry Houlden
May 5, 2021·Otolaryngology--head and Neck Surgery : Official Journal of American Academy of Otolaryngology-Head and Neck Surgery·María CostalesRubén Cabanillas
Jun 8, 2021·Clinical Neurophysiology Practice·David Burke, G Michael Halmagyi

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