多巴反应性肌张力障碍15例临床分析

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15例DRD患者的临床资料进行回顾性分析。结果平均发病年龄11．5岁，平均病程8．9年，均为慢性起病，首发症状多从下肢肌张力障碍开始，症状有明显的昼间波动，有晨轻暮重现象。小剂量多巴制剂有显著而持久的疗效。结论本病是一种较为罕见的遗传性运动障碍性疾病，小剂量多巴制剂疗效显著；对儿童期起病的肌张力障碍性患者，应考虑DRD的可能性，早期诊治，预后良好。

Clinical analysis of 15 cases with dopa-responsive dystonia

Objective To explore the pathogeny, clinical characteristics and therapy of dopa-responsive dystonia(DRD). Methods The clinical data of 15 cases with DRD were analyzed retrospectively. Results The average onse t age was 11.5 years old and the average course was 8.9 years. All these patient s developed slowly. The initial symptom was always dystonia of inferior limbs. A ll patients had notable diurnal fluctuation of the symptoms, characterized by ag gravation towards the evenings and alleviation in the mornings after sleep. Levo dopa had significant effect on the improvement of their symptoms of all these pa tients. Conclusion Dopa-responsive dystonia, a rare hereditary movement dis order, can be remarkably improved with a small-dose levodopa, especially the one s with child-onset. The early treatment is highly recommended for better prognos is.