Citation
W. Bruck et al., Myelinopathia centralis diffusa (vanishing white matter disease): Evidenceof apoptotic oligodendrocyte degeneration in early lesion development, ANN NEUROL, 50(4), 2001, pp. 532-536
Citations number
20
Categorie Soggetti
Neurology,"Neurosciences & Behavoir
Journal title
ANNALS OF NEUROLOGY
ISSN journal
03645134
→ ACNP
Volume
50
Issue
4
Year of publication
2001
Pages
532 - 536
Database
ISI
SICI code
0364-5134(200110)50:4<532:MCD(WM>2.0.ZU;2-O
Abstract
We describe histopathological changes in a 2-year-old boy who died from mye
linopathia centralis diffusa. Despite extensive white matter destruction, s
urprisingly high numbers of oligodendrocytes expressing proteolipid protein
mRNA were detected. In an active demyelinating lesion in the brainstem, ol
igodendrocytes showed typical signs of apoptosis. We suggest that death of
mature oligodendrocytes is the critical event in the disease.