Pathogenesis of cortical dysplasia caused by neuroadapted strain of mumps virus

皮质发育不良的发病机制进行了分析，通过检查由腮腺炎病毒的Kilham株感染在仓鼠神经元迁移期间产生的脑病变，腮腺炎病毒抗原检测脑室区的神经上皮细胞，侧脑室的脉络丛，和波形蛋白免疫反应放射状胶质纤维。主要病理改变为脑出血、神经元坏死、大脑皮质微沟和大脑皮层全层裂隙形成。在这些实验中看到的裂缝由胚胎成分如神经上皮细胞和生殖细胞排列。基于这些结果和Yakovlev和沃兹沃斯的原始定义，提出了以下两个结论。首先，腮腺炎病毒定位于脑室区和放射状胶质纤维内的神经上皮细胞，可能会诱导破坏性过程和随后的异常神经元迁移，导致裂缝形成。第二，延伸到软脑膜表面的室裂的形成是必要的，它应该在皮质内折叠出现之前完成，以产生与软脑膜室管膜缝相关的中脑畸形的特征性裂。

The pathogenesis of cortical dysplasia was analyzed by examining the brain lesions produced by the infection of the Kilham strain of mumps virus during the period of neuronal migration in hamsters.Mumps virus antigen was detected in the neuroepithelial cells within the ventricular zone, the choroid plexus in the lateral ventricles, and vimentin-immunoreactive radial glial fibers. The main pathological findings were cerebral hemorrhage, neuronal necrosis, microsulci on the cerebral cortex, and cleft formation through the entire thickness of the cerebral mantle. The clefts seen in these experiments were lined by embryonal elements such as neuroepithelial cells and germinal cells. Based on these results and the original definition by Yakovlev and Wadsworth, the following two conclusions were suggested. First, the mumps virus localized to the neuroepithelial cells within the ventricular zone and the radial glial fibers may induce a destructive process and subsequent anomalous neuronal migration, resulting in cleft formation. Second, the formation of the ventricular cleft extending to the pial surface, which should be complete before the cortical infolding appears, is necessary in order to produce the characteristic cleft in schizencephaly which is associated with the pial-ependymal seam.