Myelinopathia centralis diffusa (vanishing white matter disease): Evidence of apoptotic oligodendrocyte degeneration in early lesion development
Version of Record online: 28 AUG 2001
Copyright © 2001 Wiley-Liss, Inc.
Annals of Neurology
Volume 50, Issue 4, pages 532–536, October 2001
How to Cite
Brück, W., Herms, J., Brockmann, K., Schulz-Schaeffer, W. and Hanefeld, F. (2001), Myelinopathia centralis diffusa (vanishing white matter disease): Evidence of apoptotic oligodendrocyte degeneration in early lesion development. Ann Neurol., 50: 532–536. doi: 10.1002/ana.1227
- Issue online: 8 OCT 2001
- Version of Record online: 28 AUG 2001
- Manuscript Accepted: 23 JUN 2001
- Manuscript Received: 2 APR 2001
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.