Myelinopathia centralis diffusa (vanishing white matter disease):: Evidence of apoptotic oligodendrocyte degeneration in early lesion development
Myelinopathia centralis diffusa (vanishing white matter disease):: Evidence of apoptotic oligodendrocyte degeneration in early lesion development
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DOI：
10.1002/ana.1227
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发表时间：
2001-10-01
影响因子：
11.2
通讯作者：
Hanefeld, F
中科院分区：
文献类型：
--
作者：
Brück, W;Herms, J;Hanefeld, F
我们描述了一个2岁的男孩谁死于弥漫性中枢性髓鞘病变的组织病理学变化。尽管广泛的白色物质的破坏，令人惊讶的高数量的少突胶质细胞表达蛋白脂质蛋白mRNA的检测。在脑干的活动性脱髓鞘病变中，少突胶质细胞表现出典型的凋亡迹象。我们认为，成熟的少突胶质细胞的死亡是疾病的关键事件。
We describe histopathological changes in a 2-year-old boy who died from myelinopathia centralis diffusa. Despite extensive white matter destruction, surprisingly high numbers of oligodendrocytes expressing proteolipid protein mRNA were detected. In an active demyelinating lesion in the brainstem, oligodendrocytes showed typical signs of apoptosis. We suggest that death of mature oligodendrocytes is the critical event in the disease.