Ohtahara S
Brain Dev. 1984;6(6):509-19.
Of various topics concerning convulsive disorders in children, long-term prognosis of childhood epilepsy and developmental aspects of age-dependent epileptic encephalopathy (ADEE) were described. Recent progress in epileptology and introduction of effective antiepileptic drugs has allowed marked improvement in the prognosis of epilepsy. According to our long-term observation of epileptic children over 10 years, the remission rate for over three years was as high as 81.7%. Intractable cases were notably high in the West and Lennox syndromes. One of the main targets of child epileptology is ADEE, i.e. the West and Lennox syndromes in addition to early-infantile epileptic encephalopathy with suppression-burst (EIEE). The concept and categorization of ADEE were outlined with special reference to the developmental aspects of EIEE by a long-term follow-up study. Six of 10 cases of EIEE evolved into the West syndrome at two to six months of age, and two cases showed further transition to the Lennox syndrome at one year one month and three years one month of age. In accordance with the evolutional change in clinical seizure pattern, EEG showed an evolutional change from suppression-burst to hypsarhythmia and further development to diffuse slow spike-waves. These facts suggest a close relationship among these three types of ADEE and the significance of developmental aspects in the study of epilepsy.
在关于儿童惊厥性疾病的诸多主题中，描述了儿童癫痫的长期预后以及年龄依赖性癫痫性脑病（ADEE）的发育方面。癫痫学的最新进展以及有效抗癫痫药物的引入使癫痫的预后有了显著改善。根据我们对癫痫患儿超过10年的长期观察，三年以上的缓解率高达81.7%。难治性病例在韦斯特综合征和伦诺克斯综合征中尤为常见。儿童癫痫学的主要目标之一是ADEE，即除了具有暴发抑制的早期婴儿癫痫性脑病（EIEE）之外的韦斯特综合征和伦诺克斯综合征。通过长期随访研究，特别参考EIEE的发育方面，概述了ADEE的概念和分类。10例EIEE中有6例在2至6个月大时演变为韦斯特综合征，2例在1岁1个月和3岁1个月时进一步转变为伦诺克斯综合征。根据临床发作模式的演变，脑电图显示出从暴发抑制到高峰失律并进一步发展为弥漫性慢棘波的演变。这些事实表明这三种类型的ADEE之间存在密切关系，以及发育方面在癫痫研究中的重要性。
