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目的根据儿童失神癫(CAE)的临床及视频脑电图(VEEG)特点,探讨CAE的诊断标准。方法以“失神发作”及“3 Hz全导棘慢波”为关键词,检索本院VEEG数据库,通过阅读病例资料排除其他典型失神发作相关癫综合征,将临床和EEG特点符合1989年国际抗癫联盟(ILAE)CAE诊断标准的患儿,进一步采用2005年Panayiotopoulos提出的CAE诊断标准进行评估。结果共37例患儿符合1989年ILAE的CAE诊断标准,发病年龄3～11岁;失神发作频率5～60次/d;伴全面强直阵挛发作2例(5.4%)。过度换气试验诱发失神发作阳性率100%。患儿VEEG均监测到失神发作,仅表现为单纯失神7例(18.9%),仅表现为复杂失神25例(67.6%),既有单纯失神又有复杂失神5例(13.5%);同期EEG为双侧对称同步的3 Hz棘慢波暴发,持续时间3～40 s,其中4～20 s 33例,4例(10.8%)发作期有双棘慢波。发作间期29例(78.4%)睡眠期有全导棘慢波(GSW)片段发放,部分于困倦时(10例)或清醒时(9例)亦有GSW片段发放,GSW多不规则、不对称;17例(45.9%)发作间期有多棘慢波;22例(56.8%)有局灶放电,主要位于前头部;3例(8.1%)发作间期EEG未见异常。37例中仅7例(18.9%)完全符合2005年Panayiotopoulos提出的CAE诊断标准。结论符合1989年ILAE诊断标准的CAE患儿,仅有少数符合Panayiotopoulos 2005年的新诊断标准;新的CAE诊断标准过于严格,使部分患儿分类困难。
Objective To investigate the diagnostic criteria of CAE based on the clinical and video EEG features of children with absence of epilepsy (CAE). Methods With the key words of “loss of appetite” and “3 Hz all-spike and slow wave”, the VEEG database of our hospital was searched. Other typical cases of absence seizures were excluded by reading the case data. The clinical and EEG characteristics Children who met the CAE criteria of the International Anti-Seizure League (ILAE) 1989 in 1989 were further evaluated using the CAE diagnostic criteria proposed by Panayiotopoulos in 2005. Results A total of 37 children were eligible for the CAE diagnostic criteria of ILAE in 1989 with a mean age of onset of 3 to 11 years. The frequency of absence seizures was 5 to 60 times per day. Complete tonic clonic seizures occurred in 2 patients (5.4%). Hyperventilation test induced absent seizures positive rate of 100%. VEEG in children all detected absence seizures. Only 7 cases (18.9%) were simply deaf and only 25 cases (67.6%) had complicated deafness. There were 5 cases (13.5%) with simple deafness and complex dementia. EEG The 3-Hz spike and slow-wave outbreaks with symmetrical bimodal synchronization ranged from 3 to 40 s, with 33 from 4 to 20 s in 4 and 10 spikes in 4 (10.8%) episodes. There were 29 cases (78.4%) in the interictal episode with all-guide spike-and-slow wave (GSW) segment. Some GSW fragments were also distributed in drowsiness (10 cases) or awake (9 cases) Symmetrical; in 17 cases (45.9%), there were spikes and waves in the interictal period; focal discharge was found in 22 cases (56.8%), mainly in the anterior head; Only 7 of 37 (18.9%) were in full compliance with the CAE diagnostic criteria proposed by Panayiotopoulos in 2005. Conclusions Children with CAE meeting the ILAE diagnostic criteria of 1989 only met a few new diagnostic criteria of Panayiotopoulos in 2005. The new CAE diagnostic criteria were too strict and some children were difficult to classify.