| leukodystrophy with diffuse Rosenthal fibre formation | A metabolic disorder whose onset can be in infancy, adolescence, or adulthood; characterised pathologically by widespread cerebral demyelination with astrocyte and primitive oligodendroglial cell proliferation; refractile Rosenthal fibres result from the degeneration of these proliferating cells; aetiology unknown, but possibly due to a metabolic defect of astrocytes; sex-linked recessive disorder. (05 Mar 2000) |
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