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多巴反应性肌张力障碍临床资料分析
引用本文:王雪贞,薛峥,孙昌兰,方思羽. 多巴反应性肌张力障碍临床资料分析[J]. 神经损伤与功能重建, 2007, 2(4): 224-226
作者姓名:王雪贞  薛峥  孙昌兰  方思羽
作者单位:华中科技大学同济医学院附属同济医院神经内科,武汉,430030;华中科技大学同济医学院附属同济医院神经内科,武汉,430030;华中科技大学同济医学院附属同济医院神经内科,武汉,430030;华中科技大学同济医学院附属同济医院神经内科,武汉,430030
摘    要:目的:回顾多巴反应性肌张力障碍(dopa-responsive dystonia,DRD)的临床资料,以加深对该病的认识。方法:总结并分析4例DRD患者的临床表现、辅助检查及治疗情况。结果:小剂量多巴制剂(美多巴62.5 mg,每天3次)对4例患者均有明显疗效。结论:DRD是一种较为罕见的遗传性运动障碍疾病,小剂量多巴制剂有显著、持续疗效,早期治疗效果好,需与其他肌张力障碍相鉴别,以震颤首发时易误诊为少年型帕金森病。

关 键 词:多巴反应性肌张力障碍  临床特点  鉴别诊断  少年型帕金森病
文章编号:1001-117X(2007)04-0224-03
修稿时间:2007-05-29

Clinical Analysis of 4 patients with Dopa-responsive Dystonia
WANG Xue-zhen,XUE Zheng,SUN Chang-lan,FANG Si-yu. Clinical Analysis of 4 patients with Dopa-responsive Dystonia[J]. Neural Injury and Functional Reconstruction, 2007, 2(4): 224-226
Authors:WANG Xue-zhen  XUE Zheng  SUN Chang-lan  FANG Si-yu
Affiliation:Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong Univercity of Science and Technology, Wuhan 430030, China
Abstract:Objective:The clinical data of dopa-responsive dystonia(DRD) has been reviewed.Methods: Clinical manifestation and therapy of 4 patients with DRD have been summarized.Results: Four patients with DRD were well responsive to small doses of dopa-preparation(6.25 mg of madopar three times a day).Conclusion: As an infrequently inherited motor disorder,DRD must be differentiated from other motor diseases,such as juvenile Parkinsonism who presents with tremor first.The disorder responds well to small doses of dopa-preparation in earlier period of course.
Keywords:dopa-responsive dystonia  clinical manifestation  differential diagnosis  juvenile parkinsonism
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