发作性运动性舞蹈徐动症12例临床特征探讨  

Inquiry into the Clinical Characteristics of 12 Patients with Paroxysmal Kinesigenic Choreathetosis

作　　者：王力英[1] 朵振顺[1] 房晓芬[1] 乔宝风[1] 李萍[1] 

机构地区：[1]山西医科大学第一医院

出　　处：《中西医结合心脑血管病杂志》2004年第11期643-644,共2页Chinese Journal of Integrative Medicine on Cardio-Cerebrovascular Disease

摘　　要：目的　研究发作性运动性舞蹈徐动症的临床特征与治疗反应。方法　对 12例发作性运动性舞蹈徐动症病人进行了临床观察及脑电图录像、头颅CT/MRI检查。结果　 12例均无明显家族史 ,其临床表现为发作性一侧或双侧肢体、躯干不自主运动 ,可伴构音障碍。诱发因素为突然运动、紧张、惊吓等。持续时间为 2s～2 0s ,大多不超过 2min。发作频率每天 10余次 ,或数日 1次。发作时意识清楚 ,发作间期完全正常。其电生理和影像学检查无明显异常。卡马西平类药物治疗有满意疗效。结论　发作性运动性舞蹈徐动症是一种由运动诱发的、短暂的、发作性局部或全身不随意运动 ,属离子通路疾病 ,用小剂量抗癫痫药疗效好。Objective Inquiry into the clinical characteristics and treatment respond of patients with paroxysmal kinesigenic choreoathetosis (PKC).Methods 12 patients with PKC were studied by the mean of clinical observation ,video-EEG and brain CT/MRI examination.Results 12 patients,composed by 8 men and 4 women ,with the age onset of the condition from 7 to 18 years, 12.5 years in mean,no family history.It is characterized by paroxysmal involuntary movements of one side or both sides of the limbs,trunks and head, often accompanied with dysarthria .The paroxysms can be precipitated by sudden movement ,stress and being frightened. Usually, It lasts 2-20 seconds each time and never more than 2 minutes. Its frenquce is more than 10 times a day ,or once every several days.During the attack the consciousness was kept and during the intermittent period, they may be completely “healthy'. Laboratory tests including video-EEG monitoring, brain CT/MRI showes no abnormality,All patients respond well to carbamazepine and have a benign course.Conclusion PKC is characterized by short paroxysms of focal or general involuntary movement induced by Sudden movements.It is a disease due to lesions of ion passageway. Antiepilepsy drugs are effective in treating this disease

关 键 词：发作性运动性舞蹈徐动症 临床特征 疗效 构音障碍 双侧 不自主运动 运动诱发 MRI检查 治疗反应 抗癫痫药 

分 类 号：R742.2[医药卫生—神经病学与精神病学] R743[医药卫生—临床医学]