多巴反应性肌张力障碍10例分析

Analysis of 10 cases of dopa-responsive dystonia

目的认识多巴反应性肌张力障碍的临床特征,以期早期诊治,并加深对该病的认识。方法对10例DRD患者进行临床分析。结果10例患者,男3例,女7例,发病年龄4~28岁,平均12岁,首发症状均为肌张力障碍,下肢起病者8例,上肢起病2例,全部病人均有晨轻暮重的现象,给予美多巴62.5~125mg/d治疗有显著疗效。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。

Objective To understand the multi-dopa-responsive dystonia ctinicat features, with a view to early diagnosis and treatment as well as to enhance awareness of the disease. Methods Ten cases of patients with DRD were analyzed. Results Ten patients, three cases of men and 7 cases of women, onset age 4 ~ 28 years old, average age 12 years old, the first symptoms were dystonia, onset of lower extremity in 8 cases, 2 cases of upper limb onset, all patients with morning light weight were the phenomenon of the morning, Madopar was 62.5 ~ 125mg/d, and treatment effect was significant. Conclusion DRD is a rare hereditary motor disorders disease, low-dose levodopa has significant and sustained efficacy, the effect is good by early treatment, and it should be with other dystonia.