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

2001 | journal article. A publication with affiliation to the University of Göttingen.

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​Myelinopathia centralis diffusa (vanishing white matter disease): Evidence of apoptotic oligodendrocyte degeneration in early lesion development​
Bruck, W. W.; Herms, J.; Brockmann, K.; Schulz-Schaeffer, W. J. & Hanefeld, F.​ (2001) 
Annals of Neurology50(4) pp. 532​-536​.​ DOI: https://doi.org/10.1002/ana.1227 

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Authors
Bruck, Wolfgang W.; Herms, J.; Brockmann, Knut; Schulz-Schaeffer, Walter J.; Hanefeld, Folker
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.
Issue Date
2001
Status
published
Publisher
Wiley-liss
Journal
Annals of Neurology 
ISSN
0364-5134

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