以晕厥为首发症状的Panayiotopoulos综合征临床及脑电图特征  

Clinical and electroencephalogram characteristics of Panayiotopoulos syndrome with syncope as initial symptom

作　　者：华爱美 罗结仪 齐霜 李冬娜[1] 李卫[1] 余建东 秦兵 HUA Aimei;LUO Jieyi;QI Shuang;LI Dongna;LI Wei;YU Jiandong;QIN Bing(Epilepsy Center,Department of Neurosurgery,First Affiliated Hospital of Jinan University,Guangzhou 510632,China;不详)

机构地区：[1]暨南大学附属第一医院神经外科癫痫中心,广州510632 [2]暨南大学附属第一医院粤港澳中枢神经再生研究院,广州510632

出　　处：《实用医学杂志》2020年第12期1638-1643,共6页The Journal of Practical Medicine

基　　金：国家自然科学基金项目(编号:81671288);国家留学基金项目(编号:201906785005);暨南大学临床医学研究院高端人才引进项目(编号:89018041)。

摘　　要：目的探讨以晕厥为首发症状的Panayiotopoulos综合征(PS)临床及脑电图特征.方法对我院癫痫中心2016年7月至2019年12月确诊为PS的30例患儿临床资料进行回顾性分析,检索对象为所有1~14岁的患儿,建立数据库、分析晕厥样癫痫发作临床特点、脑电图和影像学特征.PS纳入标准为神经精神发育正常的患儿至少出现一次自主神经发作和至少一次脑电图出现多脑区棘(慢)波或尖(慢)波.晕厥样癫痫发作定义为临床发作性事件,表现为以下两组症状群:(1)纯晕厥样癫痫发作(pure-SLES):肌张力丧失和无反应性;(2)自主神经发作伴晕厥样癫痫发作(AS+SLES).结果在30例确诊为PS的患儿中,pure-SLES组共6例,均以晕厥样癫痫发作为首发症状;AS+SLES组共24例,表现为发作性呕吐、面色苍白、面色潮红、发绀、晕厥样癫痫发作、发作性行为改变、尿便失禁.两组患儿脑电图均为多脑区放电,头颅核磁共振未见结构性异常.结论SLES是PS中较少见的发作类型,需要我们在晕厥的鉴别诊断中细致甄别,临床上若发现伴有其它自主神经发作症状、脑电图呈多脑区放电、头颅核磁共振排除结构性异常,高度提示PS综合征.Objective To investigate the clinical and electroencephalogram(EEG)characteristics of Panayiotopoulos syndrome(PS)with syncope as initial symptom.Methods The clinical data of 30 children with PS diagnosed in our hospital from July 2016 to December 2019 were retrospectively analyzed.All the children aged 1⁃14 were searched in the epilepsy center database of our hospital for establishing the records and analyzing the children′s characteristics of syncope⁃like seizure,EEG and imaging.The inclusion criteria for PS were at least one autonomic seizures(AS)in children with neurodevelopmental normal and at least one abnormal EEG showing interictal spikes or sharp located in multiple regional of the brain.Syncope⁃like epileptic seizure(SLES)was defined as paroxysmal events that manifested as the following two groups:(1)Pure syncope⁃like seizure(pure⁃SLES)characterized by atonic seizure and unresponsiveness.(2)spontaneous ictal autonomic signs and symptoms accompanied by syncope⁃like seizure(AS+SLES).Results Among the 30 children diagnosed as PS,there were 6 cases in the pure⁃SLES group,all of which had paroxysmal syncope⁃like seizure as the first symptom,and 24 cases in the AS+SLES group whose symptoms and signs included ictal vomiting,pallor,flushing,cyanosis,syncope⁃like seizure,paroxysmal behavior change,urinary and faecal incontinence.The EEG of both groups showed multiple regional discharges,and there was no structural abnormality in MRI.Conclusion SLES is a rare seizure type of PS,which needs to be carefully identified in the differential diagnosis of childhood syncope.PS would be highly suggested if any other symptoms of autonomic seizures,multiple regional discharge and normal findings in brain MRI are found.

关 键 词：晕厥 Panayiotopoulos综合征 脑电图(EEG) 

分 类 号：R742.1[医药卫生—神经病学与精神病学]