From the Departments of Neurology (J.A.P., K.P.W.), Neuroradiology (W.W.W.), and Ophthalmology (K.P.W.), University Hospital Zurich, Switzerland.
Neurology. 2013 Oct 29;81(18):1642-3. doi: 10.1212/WNL.0b013e3182a9f435.
A 75-year-old woman complained about insecure gait since age 55. Clinical examination revealed signs of cerebellar ataxia, bilateral vestibulopathy, and peripheral sensory impairment. Sensory nerve action potentials were absent. The visually enhanced vestibulo-ocular reflex (VVOR) was impaired (video on the Neurology(®) Web site at www.neurology.org, figure 1) and the diagnosis of cerebellar ataxia (figure 2) with neuropathy and bilateral vestibular areflexia syndrome (CANVAS) was made.(1) CANVAS is considered to be a recessive disorder with a mean age at onset of 60 years.(2) VVOR impairment is its characteristic clinical sign.(2) It can only be elicited if both smooth-pursuit eye movements and the vestibulo-ocular reflex are deficient. Normally, both are redundant at low head velocities.(2.)
一位 75 岁女性自 55 岁起就抱怨步态不稳。临床检查显示存在小脑性共济失调、双侧前庭病和周围感觉障碍的体征。感觉神经动作电位缺失。视觉增强前庭眼反射（VVOR）受损（可在 Neurology®网站 www.neurology.org 观看视频，图 1），并诊断为小脑性共济失调（图 2）合并神经病和双侧前庭反射消失综合征（CANVAS）。（1）CANVAS 被认为是一种隐性疾病，发病平均年龄为 60 岁。（2）VVOR 受损是其特征性临床体征。（2）只有当平滑追踪眼球运动和前庭眼反射均缺失时才能引出。通常，在低头部速度时，两者都是冗余的。（2）
