Sadleir Lynette G, Scheffer Ingrid E, Smith Sherry, Carstensen Bendix, Carlin John, Connolly Mary B, Farrell Kevin
Department of Pediatrics, Wellington School of Medicine, University of Otago, Wellington, New Zealand.
Epilepsia. 2008 Dec;49(12):2100-7. doi: 10.1111/j.1528-1167.2008.01708.x. Epub 2008 Jun 26.
The clinical features of absence seizures in idiopathic generalized epilepsy have been held to be syndrome-specific. This hypothesis is central to many aspects of epilepsy research yet has not been critically assessed. We examined whether specific factors such as epilepsy syndrome, age, and state determine the features of absence seizures.
Children with newly presenting absence seizures were studied using video electroencephalography (EEG) recording. We analyzed whether a child's epilepsy syndrome, age, state of arousal, and provocation influenced specific clinical features of their absence seizures: duration, eyelid movements, eye opening, and level of awareness during the seizure.
Seizures (509) were evaluated in 70 children with the following syndromes: Childhood absence epilepsy (CAE), 37; CAE plus photoparoxysmal response (PPR), 10; juvenile absence epilepsy (JAE), 8; juvenile myoclonic epilepsy (JME), 6; unclassified, 9. Seizure duration was associated with epilepsy syndrome as children with JME had shorter seizures than in other syndromes, independent of age. Age independently influences level of awareness and eye opening. Arousal or provocation affected all features except level of awareness. Specific factors unique to the child independently influenced all features; the nature of these factors has not been identified.
The view that the clinical features of absence seizures have syndrome-specific patterns is not supported by critical analysis. We show that confounding variables profoundly affect clinical features and that syndromes also show marked variation. Variation in clinical features of absence seizures results from a complex interaction of many factors that are likely to be genetically and environmentally determined.
特发性全身性癫痫失神发作的临床特征被认为具有综合征特异性。这一假说在癫痫研究的许多方面都处于核心地位，但尚未得到严格评估。我们研究了癫痫综合征、年龄和状态等特定因素是否决定失神发作的特征。
对新出现失神发作的儿童进行视频脑电图（EEG）记录研究。我们分析了儿童的癫痫综合征、年龄、觉醒状态和诱发因素是否会影响其失神发作的特定临床特征：发作持续时间、眼睑运动、睁眼情况以及发作期间的意识水平。
对70名患有以下综合征的儿童的509次发作进行了评估：儿童失神癫痫（CAE），37例；CAE伴光阵发性反应（PPR），10例；青少年失神癫痫（JAE），8例；青少年肌阵挛癫痫（JME），6例；未分类，9例。发作持续时间与癫痫综合征有关，因为JME儿童的发作比其他综合征的儿童短，且与年龄无关。年龄独立影响意识水平和睁眼情况。觉醒或诱发因素影响除意识水平外的所有特征。儿童特有的特定因素独立影响所有特征；这些因素的性质尚未确定。
失神发作的临床特征具有综合征特异性模式的观点未得到批判性分析的支持。我们表明，混杂变量深刻影响临床特征，且综合征也表现出明显差异。失神发作临床特征的差异是由许多可能由遗传和环境决定的因素复杂相互作用导致的。
