单纯发作性运动诱发性运动障碍与发作性运动诱发性运动障碍伴随癫痫发作患者临床鉴别与诊治  

Clinical characteristics,diagnosis and treatment of pure paroxysmal kinesigenic dyskinesia and complicated type with epilepsy

作　　者：郝文思 韩佳琦 马芮 张夏婷[1] 高乐虹[1] 卫华[1] 林一聪[1] 陈佳 王玉平[1] Hao Wensi;Han Jiaqi;Ma Rui;Zhang Xiating;Gao Lehong;Wei Hua;Lin Yicong;Chen Jia;Wang Yuping(Department of Neurology,Xuanwu Hospital,Capital Medical University,Beijing 100053,China)

机构地区：[1]首都医科大学宣武医院神经内科,北京100053

出　　处：《中华神经科杂志》2024年第9期951-958,共8页Chinese Journal of Neurology

基　　金：国家科技资源共享服务平台计划(YCZYPT[2019]06)。

摘　　要：目的:总结单纯发作性运动诱发性运动障碍(PKD)患者与PKD伴发癫痫患者的临床特征、脑电图和脑磁图表现,以更好地鉴别两者并指导治疗。方法:回顾性分析2000—2023年于首都医科大学宣武医院门诊就诊的200例临床诊断为PKD的患者资料,根据是否伴有癫痫发作,将其分为单纯PKD(174例)与PKD伴随癫痫(26例)两组。比较两组患者在临床特征、药物治疗、脑电图和脑磁图方面的差异。结果:两组患者的临床特征基本类似,其中单纯PKD组的PKD运动障碍由情绪紧张诱发者比例(54/174,31.03%)比PKD伴随癫痫组(2/26,7.69%)高(χ^(2)=5.010, P=0.025)。在药物治疗反应方面,两组均以卡马西平为最常用的药物,但PKD伴随癫痫患者可能需要更高的治疗剂量(0.2~0.4 g/d,并逐渐加量至0.8 g/d)才能同时有效控制运动障碍及癫痫发作。在脑电图和脑磁图特征方面,PKD伴随癫痫患者脑电图异常比例更高,主要表现为局灶性的棘波/尖波[1/70(1.43%)比9/21(42.86%),χ^(2)=24.268, P<0.001],并更容易发现脑磁图的异常放电(4/18比3/5,χ^(2)=1.155, P=0.282)。两者的脑磁图偶极子均主要分布于额叶、中央区附近脑区。 结论:单纯PKD与PKD伴发癫痫患者的运动症状临床表现相似,且卡马西平仍是两者最有效的治疗手段。PKD伴随癫痫患者脑电图异常比例更高,以局灶性的棘波/尖波为主,且更容易发现脑磁图的异常放电,可用以鉴别两者。Objective:To summarize the clinical features,electroencephalogram(EEG)and magnetoencephalogram(MEG)of patients with pure paroxysmal kinesigenic dyskinesia(PKD)and PKD with epilepsy,so as to better distinguish them and guide the treatments.Methods:The clinical data of 200 patients diagnosed with PKD in the Outpatient Department of Xuanwu Hospital,Capital Medical University from 2000 to 2023 were analyzed retrospectively.The patients were divided into 2 groups:pure PKD(174 cases)and PKD with epilepsy(26 cases)according to whether accompanied by epilepsy.The differences in clinical features,drug therapy,EEG and MEG were compared between the 2 groups.Results:The clinical features of the 2 groups were essentially similar,and the proportion of PKD dyskinesia induced by emotional stress in the pure PKD group(54/174,31.03%)was higher than that in the PKD with epilepsy group(2/26,7.69%;χ^(2)=5.010,P=0.025).In terms of pharmacological response,carbamazepine was the most commonly used medication in both groups,but patients with PKD with epilepsy may need a higher therapeutic dosages(0.2-0.4 g/d,and gradually increased to 0.8 g/d)to effectively manage both dyskinesia and seizures.Regarding the EEG and MEG,the proportion of EEG abnormalities was higher in PKD patients with epilepsy,mainly manifested as focal spikes[1/70(1.43%)vs 9/21(42.86%),χ^(2)=24.268,P<0.001],together with aberrant MEG discharge(4/18 vs 3/5,χ^(2)=1.155,P=0.282).The MEG dipoles were mainly distributed in the brain regions close to the frontal lobe and central region.Conclusions:The clinical manifestations of motor symptoms of pure PKD and PKD with epilepsy are similar,and carbamazepine remains the most effective treatment.PKD patients with epilepsy have a higher proportion of abnormal EEG,mainly manifested as focal spikes,and are more likely to show abnormal discharge of MEG,which could be used to distinguish them.

关 键 词：运动障碍 癫痫 发作 脑电描记术 脑磁图描记术 

分 类 号：R742.1[医药卫生—神经病学与精神病学] R744.8[医药卫生—临床医学]