Myelinopathia centralis diffusa (vanishing white matter disease):: Evidence of apoptotic oligodendrocyte degeneration in early lesion development

被引:36
作者
Brück, W
Herms, J
Brockmann, K
Schulz-Schaeffer, W
Hanefeld, F
机构
[1] Humboldt Univ, Dept Neuropathol, Charite, D-1086 Berlin, Germany
[2] Univ Gottingen, Dept Neuropathol, D-3400 Gottingen, Germany
[3] Univ Gottingen, Dept Neuropediat, D-3400 Gottingen, Germany
关键词
D O I
10.1002/ana.1227
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
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页码:532 / 536
页数:5
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