Department of Pediatric Neurology, CHU d'Angers, and Laboratoire Angevin de Recherche en Ingénierie des Systèmes (LARIS), Université d'Angers, 4, rue Larrey, 49000 Angers, France.
Rev Neurol (Paris). 2022 Sep;178(7):659-665. doi: 10.1016/j.neurol.2022.01.009. Epub 2022 Apr 27.
Developmental and epileptic encephalopathies are conditions where there is developmental impairment related to both the underlying etiology independent of epileptiform activity and the epileptic encephalopathy. Usually they have multiple etiologies. Therefore, long-term outcome is related to both etiology-related factors and epilepsy-related factors-age at onset of epilepsy, type(s) of seizure(s), type of electroencephalographic abnormalities, duration of the epileptic disorder. This paper focuses on long-term outcome of six developmental and epileptic encephalopathies with onset from the neonatal period to childhood: early epileptic encephalopathy with suppression bursts, West syndrome, Dravet syndrome, Lennox-Gastaut syndrome, epilepsy with myoclonic atonic seizures and epileptic encephalopathy with continuous spike and waves during slow-wave sleep including Landau-Kleffner syndrome. For each syndrome, definition, main etiologies if multiple, and long-term outcome are discussed.
发育性和癫痫性脑病是指与潜在病因相关的发育障碍,而这种病因与癫痫样活动和癫痫性脑病无关。这些疾病通常有多种病因。因此,长期预后与病因相关因素和癫痫相关因素有关,如癫痫发作的起始年龄、发作类型、脑电图异常类型、癫痫持续时间。本文重点关注从新生儿期到儿童期发病的六种发育性和癫痫性脑病的长期预后:伴有抑制性爆发的早期癫痫性脑病、West 综合征、Dravet 综合征、Lennox-Gastaut 综合征、伴有肌阵挛失神发作的癫痫和伴有睡眠中慢波棘慢波持续发放的癫痫性脑病,包括 Landau-Kleffner 综合征。对于每种综合征,本文讨论了其定义、主要病因(如果有多种病因)和长期预后。