Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS)

被引：11

作者：

Taki, Masakatsu ^{[1
]}

Nakamura, Takashi ^{[1
]}

Matsuura, Hiraku ^{[2
]}

Hasegawa, Tatsuhisa ^{[3
]}

Sakaguchi, Hirofumi ^{[1
]}

Morita, Kanako ^{[2
]}

Ishii, Ryotaro ^{[2
]}

Mizuta, Ikuko ^{[2
]}

Kasai, Takashi ^{[2
]}

Mizuno, Toshiki ^{[2
]}

Hirano, Shigeru ^{[1
]}

机构：

[1] Kyoto Prefectural Univ Med, Dept Otolaryngol Head & Neck Surg, Kyoto, Japan

[2] Kyoto Prefectural Univ Med, Dept Gerontol & Neurol, Kyoto, Japan

[3] Akashi City Hosp, Dept Otolaryngol, Akashi, Hyogo, Japan

来源：

AURIS NASUS LARYNX | 2018年 / 45卷 / 04期

关键词：

CANVAS; vHIT; VVOR;

D O I：

10.1016/j.anl.2017.10.008

中图分类号：

R76 [耳鼻咽喉科学];

学科分类号：

100213 ;

摘要：

Cerebellar ataxia with neuropathy and bilateral vestibular areflexiasyndrome(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 neurootological examinations (i.e., caloric stimulation, vHIT, and VVOR) may reveal more non Caucasian cases. (C) 2018 Elsevier B.V. All rights reserved.

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页码：866 / 870

页数：5

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