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Pathogenesis of cortical dysplasia caused by neuroadapted strain of mumps virus

流行性腮腺炎病毒神经适应株所致皮质发育不良的发病机制

基本信息

批准号：
11670757
负责人：
TAKANO Tomoyuki
金额：
$ 1.02万
依托单位：
Shiga University of Medical Science
依托单位国家：
日本
项目类别：
Grant-in-Aid for Scientific Research (C)
财政年份：
1999
资助国家：
日本
起止时间：
1999 至 2000
项目状态：
已结题

来源：
https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-11670757/
关键词：
Schizencephaly Mumps virus Kilham strain Cerebral cleft Ventricular cleft Cortical dysplasia

项目摘要

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.

通过检测腮腺炎病毒Kilham株在金黄地鼠神经元迁移过程中引起的脑损害，分析了皮质发育不良的发病机制。在脑室带内的神经上皮细胞、侧脑室脉络丛和波形蛋白免疫反应的放射状胶质纤维中检测到腮腺炎病毒抗原。主要病理表现为脑出血、神经元坏死、大脑皮层微沟及覆盖整个大脑外套膜的裂隙形成。这些实验中看到的裂隙是由胚胎元素排列的，如神经上皮细胞和生发细胞。基于这些结果和雅科夫列夫和瓦兹沃斯的原始定义，得出了以下两个结论。首先，腮腺炎病毒定位于脑室带内的神经上皮细胞和放射状胶质纤维，可能会导致破坏性过程和随后的异常神经元迁移，导致裂隙的形成。其次，脑室裂隙的形成延伸到软膜表面，在皮质内折叠出现之前，脑室裂隙的形成是必要的，以便在脑裂畸形中产生与软膜-室管膜缝相关的特征性裂隙。