Chevassus-au-Louis N, Baraban S C, Gaïarsa J L, Ben-Ari Y
INSERM U29, Paris, France.
Epilepsia. 1999 Jul;40(7):811-21. doi: 10.1111/j.1528-1157.1999.tb00786.x.
In the last decade, the recognition of the high frequency of cortical malformations among patients with epilepsy especially children, has led to a renewed interest in the study of the pathophysiology of cortical development. This field has also been spurred by the recent development of several experimental genetic and non-genetic, primarily rodent, models of cortical malformations. Epileptiform activity in these animals can appear as spontaneous seizure activity in vivo, in vitro hyperexcitability, or reduced seizure susceptibility in vitro and in vivo. In the neonatal freeze lesion model, that mimics human microgyria, hyperexcitability is caused by a reorganization of the network in the borders of the malformation. In the prenatal methylazoxymethanol model, that causes a diffuse cortical malformation, hyperexcitability is associated with alteration of firing properties of discrete neuronal subpopulations together with the formation of bridges between normally unconnected structures. In agreement with clinical evidence, these experimental data suggest that cortical malformations can both form epileptogenic foci and alter brain development in a manner that causes a diffuse hyperexcitability of the cortical network.
在过去十年中，人们认识到癫痫患者尤其是儿童中皮质畸形的高发性，这引发了对皮质发育病理生理学研究的新兴趣。该领域的发展还得益于近期几种实验性遗传和非遗传（主要是啮齿动物）皮质畸形模型的开发。这些动物的癫痫样活动可表现为体内自发癫痫活动、体外兴奋性过高或体外和体内癫痫易感性降低。在模拟人类微小脑回的新生期冷冻损伤模型中，兴奋性过高是由畸形边界处网络的重组引起的。在导致弥漫性皮质畸形的产前甲基氧化偶氮甲醇模型中，兴奋性过高与离散神经元亚群放电特性的改变以及正常情况下不相连结构之间形成桥接有关。与临床证据一致，这些实验数据表明，皮质畸形既能形成致痫灶，又能以导致皮质网络弥漫性兴奋性过高的方式改变大脑发育。
