Ohtahara S, Ohtsuka Y, Yamatogi Y, Oka E
Department of Developmental Neuroscience and Child Neurology, Okayama University Medical School, Japan.
Brain Dev. 1987;9(4):371-6. doi: 10.1016/s0387-7604(87)80110-9.
A clinico-electroencephalographic study on 14 cases of the early-infantile epileptic encephalopathy with suppression-burst (EIEE) including long-term follow-up studies for one year 8 months to 12 years 2 months disclosed the specificity of EIEE in its developmental aspects. With age, clinical evolution from EIEE to the West syndrome was observed in as many as 10 cases, among which two cases showed further transition to the Lennox-Gastaut syndrome. Electroencephalographically, suppression-burst pattern gradually began to disappear from age of 3 months and disappeared by 6 months in all the cases, transforming to hypsarhythmia in 10 cases from 2 to 6 months of age, showing further transition to diffuse slow spike-and-waves in 2 cases at one year and one month and at 3 years and one month of age, respectively. Changing pattern of EEG were classifiable into two types which strongly related to the prognosis. These findings indicated EIEE to be an independent epileptic syndrome as the earliest form of the age-dependent epileptic encephalopathy.
一项对14例早期婴儿癫痫性脑病伴暴发抑制（EIEE）的临床脑电图研究，包括为期1年8个月至12年2个月的长期随访研究，揭示了EIEE在发育方面的特异性。随着年龄增长，多达10例患者出现了从EIEE向韦斯特综合征的临床演变，其中2例进一步转变为 Lennox-Gastaut 综合征。脑电图方面，暴发抑制模式在3个月龄时逐渐开始消失，所有病例在6个月时均消失，10例在2至6个月龄时转变为高度节律失调，分别有2例在1岁1个月和3岁1个月时进一步转变为弥漫性慢棘慢波。脑电图的变化模式可分为两种类型，这与预后密切相关。这些发现表明EIEE是一种独立的癫痫综合征，是年龄依赖性癫痫性脑病的最早形式。
