Myelinopathia centralis diffusa (vanishing white matter disease): Evidence of apoptotic oligodendrocyte degeneration in early lesion development
Authors:
- 8 October 2001
- journal article
- case report
- Published by Wiley in Annals of Neurology
- Vol. 50 (4) , 532-536
Abstract
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.Keywords
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