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伴有神经病变和前庭无反射综合征(CANVAS)的小脑共济失调的前庭康复——病例报告

Vestibular rehabilitation in cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS)- A case report.

作者信息

Harrell R G, Cassidy A R, Klatt B N, Hovareshti P, Whitney S L

机构信息

School of Health and Rehabilitation Sciences, Department of Physical Therapy, University of Pittsburgh, Pittsburgh, PA, 15219, USA.

Rehabilitation Institute, University of Pittsburgh Medical Center, Pittsburgh, PA, 15219, USA.

出版信息

J Otol. 2023 Oct;18(4):199-207. doi: 10.1016/j.joto.2023.06.004. Epub 2023 Jul 20.

Abstract

BACKGROUND AND PURPOSE

Cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS) is a neurodegenerative disease of the cerebellum. The disease progression is slow, with up to 25% of people diagnosed needing to use a wheelchair after 15 years from diagnosis. Vestibular symptoms arise from centrally-mediated ocular movement degradation and the reduced vestibular-ocular reflex functioning bilaterally. To date, no report has shown an improvement in VOR gain or gait outcome measures in someone with CANVAS after a course of vestibular physical therapy.

CASE DESCRIPTION

A 65-year-old male, Patient X, first noticed symptoms in his fourth decade of life and was diagnosed with (CANVAS) in his seventh decade. Patient X reported numbness and tingling in his hands and feet, decreased ability to perform daily activities, and several falls.

INTERVENTION

Patient X completed a four-month course of vestibular physical therapy, including vestibular ocular reflex exercises, balance training, gait training, and the VestAid application for eye gaze compliance monitoring. The Vestaid application uses eyes and facial recognition software to record the percentage of time that the patient kept their eyes on the target.

OUTCOMES

After vestibular therapy, Patient X had a clinically meaningful improvement in gait speed: from 1.02 m/s to 1.13 m/s and in the Functional Gait Assessment from 20/30 to 27/30. Patient X's eye gaze compliance improved from a median of 43% (range 25-68%) to a median of 67% (58-83%).

DISCUSSION

This case study demonstrates that vestibular rehabilitation improved eye gaze compliance and functional outcomes in a person living with CANVAS.

摘要

背景与目的

小脑性共济失调、神经病和前庭反射消失综合征(CANVAS)是一种小脑神经退行性疾病。疾病进展缓慢,高达25%的确诊患者在确诊后15年需要使用轮椅。前庭症状源于中枢介导的眼球运动退化以及双侧前庭眼反射功能减退。迄今为止,尚无报告显示CANVAS患者在接受一个疗程的前庭物理治疗后,其前庭眼反射增益或步态结果指标有所改善。

病例描述

一名65岁男性,X患者,在其40多岁时首次出现症状,并在70多岁时被诊断为CANVAS。X患者报告手脚麻木、刺痛,日常活动能力下降,且多次跌倒。

干预措施

X患者完成了为期四个月的前庭物理治疗课程,包括前庭眼反射练习、平衡训练、步态训练以及用于眼注视依从性监测的VestAid应用程序。VestAid应用程序使用眼睛和面部识别软件记录患者注视目标的时间百分比。

结果

前庭治疗后,X患者的步态速度有了具有临床意义的改善:从1.02米/秒提高到1.13米/秒,功能步态评估从20/30提高到27/30。X患者的眼注视依从性从中位数43%(范围25 - 68%)提高到中位数67%(58 - 83%)。

讨论

本病例研究表明,前庭康复改善了CANVAS患者的眼注视依从性和功能结果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7c9c/10593570/368db2eb70d3/gr1a.jpg

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