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

@article{Brck2001MyelinopathiaCD,
  title={Myelinopathia centralis diffusa (vanishing white matter disease): evidence of apoptotic oligodendrocyte degeneration in early lesion development.},
  author={Wolfgang Br{\"u}ck and Jochen Herms and Knut Brockmann and Walter J. Schulz-Schaeffer and Folker A Hanefeld},
  journal={Annals of neurology},
  year={2001},
  volume={50 4},
  pages={532-6}
}
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.