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原发性成年发病眼睑痉挛和痉挛性斜颈的发病年龄和症状扩散。

Age at onset and symptom spread in primary adult-onset blepharospasm and cervical dystonia.

机构信息

Department of Neurosciences and Sensory Organs and School of Motor Sciences, Aldo Moro University of Bari, Bari, Italy.

出版信息

Mov Disord. 2012 Sep 15;27(11):1447-50. doi: 10.1002/mds.25088. Epub 2012 Aug 13.

Abstract

BACKGROUND

The site of dystonia onset is known to affect the risk of spread in primary adult-onset focal dystonia, but other factors possibly influencing spread are unknown. This study explored the relationship between age and spread of dystonia in primary adult-onset focal dystonia.

METHODS

Two survival models analyzed spread of dystonia in a large cohort of patients with primary blepharospasm (BSP) and cervical dystonia. The first model was based on time interval between onset and spread of dystonia, and the second model was based on age at spread.

RESULTS

Patients presenting with BSP had a 2-fold higher rate of spread than those presenting with cervical dystonia, regardless of the survival model used. However, survival analysis, based on age at spread, showed that spread develops at a similar age period in both groups, with most spread events occurring after the age of 50.

CONCLUSIONS

The convergent age of spread in BSP and cervical dystonia is a novel finding indicating age as a factor modulating spread of dystonia. These findings may assist in informing prognostication for patients with primary adult-onset focal dystonia.

摘要

背景

局灶性肌张力障碍发病部位已知会影响原发性成人发病局灶性肌张力障碍的扩散风险,但其他可能影响扩散的因素尚不清楚。本研究探讨了原发性成人发病局灶性肌张力障碍中年龄与肌张力障碍扩散之间的关系。

方法

两种生存模型分析了原发性眼睑痉挛(BSP)和颈肌张力障碍患者中大量患者的肌张力障碍扩散情况。第一个模型基于发病和肌张力障碍扩散之间的时间间隔,第二个模型基于扩散时的年龄。

结果

无论使用哪种生存模型,与颈肌张力障碍患者相比,出现 BSP 的患者扩散的发生率都高出两倍。然而,基于扩散年龄的生存分析表明,两组的扩散发生在相似的年龄阶段,大多数扩散事件发生在 50 岁以后。

结论

BSP 和颈肌张力障碍的扩散年龄趋同是一个新的发现,表明年龄是调节肌张力障碍扩散的因素之一。这些发现可能有助于为原发性成人发病局灶性肌张力障碍患者提供预后信息。

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