发作性运动诱发肌张力障碍的临床与视频脑电图研究

CLINICAL CHARACTERISTICS AND VIDEO ELECTROENCEPHALOGRAM STUDY OF PAROXYSMAL KINESIGENIC DYSTONIA

[目的]探讨发作性运动诱发肌张力障碍(paroxysmalkinesigenicdystonia,PKD)的临床和视频脑电图特征。[方法]观察3例PKD患者的临床和视频脑电图特点,进行头颅核磁共振检查,同时使用卡马西平治疗并观察其转归。[结果]3例患者共观察到15次发作,表现为发作性一侧或双侧肢体的肌张力障碍和舞蹈症,持续10～20s,均有明显的诱发因素:从坐位起立或从静止开始行走、接受询问、受惊吓和接电话,发作时无意识障碍。24hVEEG检查未见癫痫样放电。头颅核磁共振阴性。短期随访卡马西平治疗有效。[结论]PKD是以运动诱发的表现为短暂性舞蹈样手足徐动症等肌张力障碍为特征的良性疾病,脑电图多无异常,卡马西平能有效控制其发作。

[ Objective] To approach the clinical and video electroencephalogram （VEEG） characteristics of patients with paroxysmal kinesigenic dystonia （PKD） . [Methods] The clinical features and video electroencephalogram characteristics of three patients with PKD were observed. Magnetic resonance imagings were done in all the patients. Curative effects of earbamazepine were also folhwed up. [Flosults] There were 15 onsets in all the three cases.The clinical characteristics of PKD were dystonie posture and chorea which lasted usually 10 to 20 seconds each time. Patients with PKD were liable to attacks provoked by sudden movement, changing posture, inquire, startle and answering the phone.Moreover, patients never lost their consciousness. Epileptiform discharges were not observed in VEEG lasting 24 hours. Neuroimaging examination showed no abnormality.All cases responded favorably to earbamazepine. [Gonclusion] PKD is characterized by movement induced, transient, and involuntary movement, which is a kind of innocuousness disease. EEG of patients with PKD are almost normal and earbamazepine is effective.