摘要
目的应用多项电生理技术观测婴儿痉挛(IS)的临床发作特征,探索痉挛、肌阵挛和强直性发作的临床-电生理鉴别要点。方法应用视频脑电-肌电多导记录及抽搐逆向锁定的脑电平均技术(jerk-loeked back averaging,JLA),对8例1岁以下 IS 婴儿的681次临床明显发作(包括经测试最后被摒除的20次非皮层性肌阵挛),进行临床和多项电生理的实时联合记录分析。同时以其中1例 Aicardi 综合征 IS 临床发作前2个月的58次肌阵挛发作为对照。结果 8例 IS 共记录到3种发作形式,包括痉挛、肌阵挛和强直性发作,分别占94.4%(624/661次)、4.5%(30/661次)和1.1%(7/661次)。痉挛发作多为轴性肌群收缩,具有丛集性及"渐强-渐弱"特征,85.7%(535/624次)的痉挛发作时间在0.4～3.0 s 间,14.3%(89/624次)持续3～7 s。此外,还根据发作期 EEG 认定273次微小痉挛发作。发作期脑电图多为全脑性高幅慢波或低-中幅快波活动(89.1%),经 JLA 叠加后发作期脑电与肌电间未见明确锁时关系。肌阵挛为闪电样的肌肉收缩,发作时间<400 ms,发作期脑电图或为痫性放电,或无明显改变。经 JLA 叠加后证实痫性肌阵挛有皮层放电波,且与肌电之间有恒定锁时关系。强直性发作持续3 s 以上,脑电图为低幅快波及中幅θ活动。此外,根据强直痉挛的强直达峰时间<2 s、发作期 EEG 及轴性发作与 IS 一致等特点,可与强直性发作区别。结论 IS 临床以痉挛发作为主,借助多导电生理记录及 JLA 等电生理技术,能满意地对痉挛、强直性痉挛、肌阵挛和强直性发作等婴幼儿期常见癫痫发作进行鉴别,从而有助对婴儿期癫痫的正确诊断、分类与治疗。
Objective To explore the characteristics of various seizure types in infantile spasm （IS） and to recognize the clinical and electrophysiolagical differences among spasm, myoclonic and tonic seizures. Methods Totally 681 seizures of 8 infants with IS were analyzed, including 20 episodes of noncortical myoclonus which were finally ruled out by video-electroencephalogram-electromyogram polygraphic recordings （VEEG-EMG） and off-line analysis of jerk-locked back averaging （JLA）. As a control, the data of 58 myoclonic seizures collected from an infant with Aicardi syndrome within two months before his typical clinical presentations of IS were also analyze＆ Results Three types of seizures were recorded from the 8 infants, including spasm, myoclonic and tonic seizures with the incidence of 94.4% , 4. 5%, and 1.1%, respectively. Spasms were mostly presented as body muscle contraction axially, which often occurred in clusters and evolved in a crescendo-decrescendo manner; 85.7% of them lasted for 0. 4 -3.0 s and 14. 3% for 3 -7 s. In addition, there were 273 seizures which were identified as subtle spasms according to their ictal EEG with high voltage slow wave （HVS） and fast wave bursts in most. There was no constantly timelocked EEG correlating to spasms even when JLA was applied for analysis. Myoclonic seizures were shocklike muscle constraction lasting for less than 400 ms with or without visible epileptic discharges in its ictal EEG. However, there was a time-locked cortical discharge discerned by JLA in epileptic myoclonus. Tonic seizures were consisted of sustained muscle contractions involving limbs and trunk, lasting for more than 3 s. Its ictal EEGs were more likely low amplitude fast waves and medium amplitude 0 activities. Some spasms, named as tonic spasm, could be distinguished from tonic seizure according to the seizure duration which was always less than 2 s in tonic spasms and their different EEG patterns. Conclusions There were various seizure types in IS but spasm was the predominant on
出处
《中华儿科杂志》
CAS
CSCD
北大核心
2007年第2期109-114,共6页
Chinese Journal of Pediatrics
关键词
痉挛
婴儿
电生理学
脑电描记术
Spasms, infantile
Electrophysiology
Electroencephalography
