Bode H, Bubl R
Universitäts-Kinderspital, Basel/Schweiz.
Klin Padiatr. 1994 Jan-Feb;206(1):12-7. doi: 10.1055/s-2008-1046573.
Lissencephaly syndromes are rare disturbances of the neuronal migration with agyria and/or pachygyria. Typical patterns in the EEG of 5 children with lissencephaly are presented. Rhythmic alpha- or beta-waves with high amplitudes of diffuse or fronto-precentral localisation are characteristic in type I lissencephaly. In type II lissencephaly initially theta- or delta-waves of somewhat lower amplitude are observed. Sharp and slow wave-complexes of very high amplitude are found more often in type I lissencephaly. They seem to correlate with the severity of the brain malformation and the epilepsy. In both types of lissencephaly the spatial organisation of the background activity and the sleep-wake differentiation are absent. The EEG of most type I lissencephalies show no reactivity to intravenous benzodiazepines. With increasing age the frequency of the background activity and of the high amplitude rhythmic waves decrease in type I and increase in type II lissencephaly. A complete dedifferentiation of the EEG tracings has been observed in the course of type I lissencephaly. Lissencephaly is diagnosed by imaging techniques. However, with a high probability lissencephaly can be suspected already by its EEG.
无脑回综合征是一种罕见的神经元迁移障碍,伴有无脑回和/或巨脑回。本文展示了5例无脑回患儿脑电图的典型模式。I型无脑回的特征是出现弥漫性或额中央前局部定位的高振幅节律性α波或β波。II型无脑回最初观察到的是振幅稍低的θ波或δ波。I型无脑回更常出现振幅极高的尖波和慢波复合波。它们似乎与脑畸形的严重程度和癫痫有关。在两种类型的无脑回中,背景活动的空间组织和睡眠-觉醒分化均缺失。大多数I型无脑回患儿的脑电图对静脉注射苯二氮䓬类药物无反应。随着年龄增长,I型无脑回背景活动和高振幅节律性波的频率降低,II型无脑回则增加。在I型无脑回病程中观察到脑电图描记完全去分化。无脑回通过影像学技术诊断。然而,根据脑电图很有可能早期怀疑无脑回。