Brück W, Herms J, Brockmann K, Schulz-Schaeffer W, Hanefeld F
Department of Neuropathology, University of Göttingen, Germany.
Ann Neurol. 2001 Oct;50(4):532-6. doi: 10.1002/ana.1227.
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.
