摘要
目的 探讨多巴反应性肌张力障碍的临床特点、诊断和治疗方法。方法 回顾性分析10例多巴反应性肌张力障碍患者的临床表现、诊断方法和治疗效果。结果 多巴反应性肌张力障碍发病年龄较早,多见于女孩,表现为肌张力障碍或合并帕金森综合征,呈昼间波动,应用左旋多巴治疗有明显疗效。结论 对幼年起病的肌张力障碍或帕金森综合征患者,应首先使用左旋多巴治疗,以筛选出多巴反应性肌张力障碍,使患者能够保持正常生活质量。
Objective The clinical features, diagnostic and therapeutic methods in dopa-responsive dystonia (DRD) were studied. Methods Systematic study of clinical features, diagnosis and therapeutic effect in 10 patients with DRD was performed and retrospectively analyzed. Results DRD usually occurs in childhood, girls affect more often, the main manifestations include dystonia or complicated with Parkinsonism, diurnal fluctuation , well - response to L - dopa. Conclusion DRD should be primarily treated by L - dopa in childhood when onset of dystonia or Parkinsonism. The patients with such treatment will survival with normal quality of life.