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双侧苍白球刺激治疗肝豆状核变性。

Bilateral pallidal stimulation for Wilson's disease.

机构信息

Toronto Western Hospital, Toronto, Ontario, Canada.

出版信息

Mov Disord. 2013 Aug;28(9):1292-5. doi: 10.1002/mds.25446. Epub 2013 Apr 3.

Abstract

BACKGROUND

To report on the clinical efficacy of bilateral globus pallidus internus deep brain stimulation in a 29-year-old patient with severe generalized dystonia secondary to Wilson's disease.

METHODS

The primary outcome measure was the Burke-Fahn-Marsden Dystonia Scale motor severity score (blinded assessment) and the secondary outcome measures were the Abnormal Involuntary Movement Scale (blinded assessment) and the Zaritt Caregiver Burden Interview score, at 20-week postoperative follow up.

RESULTS

There was a 14% improvement in the Burke-Fahn-Marsden Dystonia Scale motor severity score. Abnormal Involuntary Movement Scale score remained unchanged while the Zaritt Caregiver Burden Interview score improved by 44.4%.

CONCLUSIONS

Bilateral globus pallidus deep brain stimulation can be effective in ameliorating dystonia and caregiver burden in Wilson's disease. Outcomes may depend on the stage of the disease at which the surgical procedure is completed. © 2013 Movement Disorder Society.

摘要

背景

报告一名 29 岁威尔逊病继发严重全身性肌张力障碍患者双侧苍白球 internus 深部脑刺激的临床疗效。

方法

主要结局指标为 Burke-Fahn-Marsden 肌张力障碍量表运动严重程度评分(盲法评估),次要结局指标为异常不自主运动量表(盲法评估)和 Zaritt 照顾者负担访谈评分,在术后 20 周随访时。

结果

Burke-Fahn-Marsden 肌张力障碍量表运动严重程度评分改善 14%。异常不自主运动量表评分保持不变,而 Zaritt 照顾者负担访谈评分改善 44.4%。

结论

双侧苍白球深部脑刺激可有效改善威尔逊病患者的肌张力障碍和照顾者负担。结果可能取决于手术完成时疾病的阶段。© 2013 运动障碍学会。

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