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原发性眼睑痉挛和颈肌张力障碍中的拮抗运动现象学。

The phenomenology of the geste antagoniste in primary blepharospasm and cervical dystonia.

机构信息

Department of Neurological and Psychiatric Sciences, University of Bari, Italy.

出版信息

Mov Disord. 2010 Mar 15;25(4):407-12. doi: 10.1002/mds.23011.

DOI:10.1002/mds.23011
PMID:20108367
Abstract

The geste antagoniste (GA), a relatively common feature of adult-onset primary dystonia, has been systematically evaluated only in cervical dystonia, but it is still unclear whether its frequency and phenomenology differ among the various forms of focal dystonia. We analysed the frequency, phenomenology, effectiveness, and relationship of the GA with demographic/clinical features of dystonia in a representative clinical series of patients with the two most common forms of adult-onset primary dystonia, blepharospasm (BSP) and cervical dystonia (CD). Clinical data were gathered using a standardized questionnaire, which showed substantial test-retest reliability (kappa = 0.79, P < 0.00001). The frequency of GA was similar among patients with BSP (42/59, 71.2%) and patients with CD (27/32, 84.4%), and in both groups GA showed similar effectiveness in reducing dystonia. The repertoire of GA was heterogenous in both BSP and CD patients, in whom seven BSP-related and five CD-related types of GA were recorded, and a "forcible" type of GA was present in 69% of BSP patients and in 48.1% of CD patients. In our whole patient population, age at dystonia onset was significantly lower among patients reporting a GA compared to those without GA (P = 0.01). GA features shared by BSP and CD predominate over differences, suggesting common mechanisms underlying this phenomenon in the two forms of primary adult-onset dystonia.

摘要

拮抗肌动作(GA)是成人发病原发性肌张力障碍的一个相对常见的特征,已经在颈肌张力障碍中进行了系统评估,但它在各种局灶性肌张力障碍中的频率和表现形式是否不同仍不清楚。我们分析了在成人发病原发性肌张力障碍的两种最常见形式,眼睑痉挛(BSP)和颈肌张力障碍(CD)的代表性临床系列患者中,GA 的频率、表现、效果及其与肌张力障碍的人口统计学/临床特征的关系。使用标准化问卷收集临床数据,该问卷显示出较高的测试-重测可靠性(kappa = 0.79,P < 0.00001)。BSP 患者(42/59,71.2%)和 CD 患者(27/32,84.4%)中 GA 的频率相似,并且在两组中 GA 都显示出相似的减轻肌张力障碍的效果。在 BSP 和 CD 患者中,GA 的动作范围是异构的,记录了七种与 BSP 相关和五种与 CD 相关的 GA 类型,并且在 69%的 BSP 患者和 48.1%的 CD 患者中存在“强制”类型的 GA。在我们的所有患者人群中,报告有 GA 的患者与没有 GA 的患者相比,肌张力障碍发病年龄明显较低(P = 0.01)。BSP 和 CD 共有的 GA 特征多于差异,表明这两种成人原发性肌张力障碍形式中存在共同的机制。

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