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Myelinopathia centralis diffusa (vanishing white matter disease): Evidence of apoptotic oligodendrocyte degeneration in early lesion development
Author(s) -
Brück Wolfgang,
Herms Jochen,
Brockmann Knut,
SchulzSchaeffer Walter,
Hanefeld Folker
Publication year - 2001
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.1227
Subject(s) - oligodendrocyte , white matter , pathology , lesion , demyelinating disease , brainstem , apoptosis , medicine , disease , myelin , biology , central nervous system , endocrinology , magnetic resonance imaging , genetics , radiology
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.