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什么是偏侧肌张力障碍?

What Is Hemidystonia?

作者信息

Vizcarra Joaquin A, Jinnah Hyder A

机构信息

Department of Neurology Emory University School of Medicine Atlanta Georgia USA.

Department of Human Genetics Emory University School of Medicine Atlanta Georgia USA.

出版信息

Mov Disord Clin Pract. 2023 Feb 3;10(3):477-481. doi: 10.1002/mdc3.13659. eCollection 2023 Mar.

Abstract

BACKGROUND

Hemidystonia is defined as dystonia restricted to one side of the body. It is traditionally believed to result from a lesion in the contralateral hemisphere.

OBJECTIVES

To describe a series of hemidystonia patients without lesions on brain imaging.

METHODS

We searched for individuals with potential hemidystonia who were included in the Dystonia Coalition or Movement Disorder Society Genetic mutation database (MDSgene), and conducted a systematic review.

RESULTS

We found 10 individuals classified as hemidystonia or with homolateral limb dystonia among 3696 cases enrolled by the Dystonia Coalition, 9 cases in MDSgene, and one idiopathic case in the literature. None had evidence of a brain lesion. Body distributions used to define hemidystonia varied considerably and were not always restricted to one side of the body.

CONCLUSIONS

Hemidystonia may be idiopathic or genetic, without any obvious brain lesion. The varied use of the term suggests the need for more specific clinical criteria to define "half the body."

摘要

背景

偏侧肌张力障碍被定义为局限于身体一侧的肌张力障碍。传统上认为它是由对侧半球的病变引起的。

目的

描述一系列脑成像无病变的偏侧肌张力障碍患者。

方法

我们在肌张力障碍联盟或运动障碍协会基因突变数据库(MDSgene)中搜索可能患有偏侧肌张力障碍的个体,并进行系统评价。

结果

我们在肌张力障碍联盟登记的3696例病例中发现10例被归类为偏侧肌张力障碍或同侧肢体肌张力障碍,在MDSgene中有9例,文献中有1例特发性病例。均无脑病变证据。用于定义偏侧肌张力障碍的身体分布差异很大,并不总是局限于身体一侧。

结论

偏侧肌张力障碍可能是特发性或遗传性的,无任何明显脑病变。该术语的不同用法表明需要更具体的临床标准来定义“身体的一半”。

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