Objective To explore the pathogeng, clinical characteristics and therapy of dopa-responsive dystonia (DRD). Methods The clinical datd of 2 cases with DRD were analyzed retrosponsive. Results Two patients with DRD were wel responsive to smal doeses of dopa preparation (6.25 mg of madopar three times a day). Conclusion DRD is a rare hereditary movement disorder,can be remarkably improved with smal-does levodopa.DRD must be dif erentiated from othere motor diseases.%目的:探讨多巴反应性肌张力障碍(DRD)的病因,临床特点及治疗,加强对该病的认识。方法对2例患者经行回顾性分析。结果小剂量多巴制剂(美多巴62.5mg,3次/d)对2例患者均有明显疗效。结论 DRD是一种较为罕见地遗传性运动障碍疾病,小剂量多巴制剂疗效显著,并需与其他肌张力障碍相鉴别。
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