特发性扭转性肌张力不全的临床及治疗

Study of clinical character,treatment of idopathic torsion dystonia in 10 children

目的 探讨小儿特发性扭转性肌张力不全的临床特征、治疗方法及疗效的初步评价。方法 对 10例特发性扭转性肌张力不全患儿的临床特征进行回顾性分析。均采用口服药物治疗 ,住院治疗 1例缓解 ,9例有效 ,并对 5例进行随访 ,平均 2年 6个月。结果 发病高峰年龄 6～ 9岁 ,单下肢起病 5例 ,单上肢 3例 ,颈部 2例。 10例实验室及头颅影像检查均正常。随访 5例坚持治疗 ,3例好转 ,2例无效。结论 药物治疗是小儿特发性扭转性肌张力不全的主要治疗方法 ,疗效有时不稳定 ,治疗需个体化。

Objective To study the clinical character, treatment and efficiency of idiopathic torsion dystonia.Method To review the clinical character of 10 children with idiopathic torsion dystonia. All patients involved used drug treatment for 27 days by average.One of them recovered, 9 cases become better.Five of them were followed up 30 months by average.Results Peak age at onset is 6～9 years. In 5 children the disturbance begins in one leg, 3 begins with one arm and 2 begins with the cervical region. All patients have normal results of laboratory and imaging investigations.Five children followed up all insisted on treated. Of 5 cases, 3 has alleviation, 2 has no alleviation than before.Conclusions Medical treatment is the main way to cure the patient with idiopathic torsion dystonia. The efficiency is not same, treatment is different according to different children.