Camfield Peter, Camfield Carol
Department of Pediatrics, Dalhousie University and the IWK Health Centre, Halifax, Nova Scotia, Canada.
Epilepsia. 2002;43 Suppl 3:27-32. doi: 10.1046/j.1528-1157.43.s.3.3.x.
We reviewed the clinical features, outcome, and treatment of many of the epileptic syndromes that begin in the childhood from 2 to 12 years of age, using a review of the literature and personal experience, with most references to authoritative texts. The developmental tasks of childhood are centered on refinement of motor skills and development of complex intellectual and social skills. The childhood onset epilepsies can be divided into benign, intermediate, and catastrophic based on their impact on childhood development. The clearest benign epilepsy is benign rolandic epilepsy, which often does not require medication treatment. The definition of benign occipital epilepsy is still often vague. In the intermediate category, childhood absence epilepsy often has associated learning disorders and a poor social outcome. About 50% of children with cryptogenic partial seizures have a very benign course, even though their epilepsy syndrome is not well defined. Generalized epilepsy with febrile seizures plus (GEFS+) has a dominant inheritance with a defined defect in cerebral sodium channels, but varies considerably in severity within affected members of the same kindred. The catastrophic epilepsies in childhood all have an inconsistent response to AED treatment and include continuous spike-wave in slow sleep (with variable severity), Landau-Kleffner syndrome (with a confusing overlap with autistic regression), the Lennox Gastaut syndrome (with broad defining features), and myoclonic-astatic epilepsy (with important overlaps with Lennox-Gastaut). Many of the epilepsies that begin in childhood are benign. Others interfere seriously with cognitive and social development.
我们通过文献回顾和个人经验,参考了多数权威文献,对许多起病于2至12岁儿童期的癫痫综合征的临床特征、转归及治疗进行了综述。儿童期的发育任务主要围绕运动技能的完善以及复杂智力和社交技能的发展。儿童期起病的癫痫根据其对儿童发育的影响可分为良性、中间型和灾难性三类。最典型的良性癫痫是良性罗兰多癫痫,通常无需药物治疗。良性枕叶癫痫的定义往往仍不明确。在中间型中,儿童失神癫痫常伴有学习障碍且社会转归不佳。约50%的隐源性部分性癫痫患儿病程非常良性,尽管其癫痫综合征尚不明确。伴有热性惊厥附加症的全身性癫痫(GEFS+)具有显性遗传,存在明确的脑钠通道缺陷,但在同一家族的受累成员中严重程度差异很大。儿童期的灾难性癫痫对抗癫痫药物治疗的反应均不一致,包括慢波睡眠期持续棘慢波(严重程度不一)、Landau-Kleffner综合征(与孤独症退行性变存在混淆的重叠)、Lennox-Gastaut综合征(具有广泛的定义特征)以及肌阵挛失张力癫痫(与Lennox-Gastaut有重要重叠)。许多起病于儿童期的癫痫是良性的。其他一些则严重干扰认知和社会发展。