Dulac O J, Chiron C
Université René Descartes, Paris, France.
Baillieres Clin Neurol. 1996 Dec;5(4):765-81.
Malignant epileptic encephalopathies in children comprise a series of age-related generalized epilepsy syndromes: neonatal myoclonic encephalopathy and early epileptic encephalopathy in the neonatal period, migrating focal seizures in early infancy, infantile spasms and West syndrome, severe myoclonic epilepsy and non-progressive myoclonic encephalopathies in infancy, and Lennox-Gastaut syndrome, myoclonic astatic epilepsy, continuous spike waves in slow sleep and Rasmussen disease in childhood. Clinical and EEG characteristics are the major diagnostic clues. Aetiology remains unknown but for the majority of the cases, a combination of different factors, particularly lesions, genetic predisposition and age-related features seem to be variously combined to produce an intractable condition.
新生儿期的新生儿肌阵挛性脑病和早期癫痫性脑病、婴儿早期游走性局灶性发作、婴儿痉挛症和韦斯特综合征、婴儿期严重肌阵挛性癫痫和非进行性肌阵挛性脑病，以及儿童期的伦诺克斯-加斯托综合征、肌阵挛失张力癫痫、慢波睡眠期持续棘慢波和拉斯穆森病。临床和脑电图特征是主要的诊断线索。病因尚不清楚，但在大多数病例中，不同因素的组合，尤其是病变、遗传易感性和年龄相关特征似乎以不同方式组合，导致病情难以控制。
