摘要
目的探讨多巴反应性肌张力障碍的病因、临床特点和治疗。方法回顾性分析36例多巴反应肌张力障碍患者的临床资料。结果 36例中男8例,女28例,其中包含2个家系。发病年龄平均9.5岁。均为缓慢起病,首发症状为始自下肢的肌张力障碍者32例,4例以帕金森病样表现起病。症状均呈晨轻暮重。头颅CT、MRI、神经电生理等检查均正常。小剂量多巴制剂治疗有明显疗效。结论本病临床特征显著,只要认识到位,诊断不难,小剂量多巴制剂治疗效果好。
Objective To investigate the pathogenesis, clinical characteristics and treatment of dopa-responsive dystonia. Methods A retrospective analysis was made on the clinical data of 36 cases of dopa-responsive dystonia. Results Of 36 patients including two families with dopa-responsive dystonia, 8 cases were male and 28 cases were female. The average onset age was 9.5 years. The first manifestation of 32 cases was dystonia in the lower limbs. The first manifestation of 4 cases was to be sim- ilar to symptoms of Parkinson. All patients had notable diurnal fluctuation of the symptoms. Investigations such as brain CT, MRI and neuroelectrophysiological examinations did not show any abnormalities. Most of patients responded well to dopa with small dosage. Conclusion Clinical characteristics of dopa-responsive dystonia is notable. The diagnosis of the disease is not dif- ficult if correct understanding of the features of the disorder can be kept in mind and proper importance attached to them. This disease has good responses to levodopa with small dosage.
出处
《中国实用神经疾病杂志》
2012年第20期20-21,共2页
Chinese Journal of Practical Nervous Diseases
关键词
多巴反应性肌张力障碍
病因
临床特点
Dopa-responsive dystonia
Pathogenesis, Clinical characteristics
