Dystonia in Wilson's disease

Dystonia in Wilson's disease
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DOI:
10.1002/mds.1118
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发表时间:
2001-07-01
期刊:
影响因子:
8.6
通讯作者:
Kostic, VS
Kostic, VS
中科院分区:
医学1区
文献类型:
--
作者:
Svetel, M;Kozic, D;Kostic, VS

文献摘要

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在27例神经系统形式的威尔逊病(WD)和优化治疗的连续患者中研究了肌张力障碍运动的频率和类型以及磁共振成像(MRI)所示的可能与肌张力障碍相关的大脑异常。肌张力障碍10例(37%),其中一半为全身性肌张力障碍,2例为节段性肌张力障碍,2例为多灶性肌张力障碍,1例为双侧足肌张力障碍。肌张力障碍是一个目前的迹象,在4名患者和发展的过程中,在6名患者的疾病,尽管管理的治疗WD。与无肌张力障碍的WD患者(24%)相比,壳核是肌张力障碍(80%)中唯一显著更频繁损伤的结构,这表明WD中该脑区异常与肌张力障碍运动之间存在关系。(C)2001运动障碍协会。
The frequency and type of dystonic movements, as well as brain abnormalities, as depicted with magnetic resonance imaging (MRI), which might correlate with dystonia, were studied in 27 consecutive patients with a neurologic form of Wilson's disease (WD) and optimized treatment. Dystonia was found in 10 patients (37%), being generalized in half of them, while two patients had segmental, two patients multifocal dystonia, and one patient bilateral foot dystonia. Dystonia was a presenting sign in four patients and developed later in the course of the disease in six patients, despite the administered therapy for WD. Putamen was the only structure significantly more frequently lesioned in dystonic (80%) in comparison to WD patients without dystonia (24%), suggesting a relation between abnormalities in this brain region and dystonic movements in WD. (C) 2001 Movement Disorder Society.