Leukodystrophy with diffuse Rosenthal fiber formation

a metabolic disorder with onset in infancy, adolescence, or adulthood; characterized pathologically by widespread cerebral demyelination with astrocyte and primitive oligodendroglial cell proliferation; refractile Rosenthal fibers result from the degeneration of these proliferating cells; etiology unknown, but possibly due to a metabolic defect of astrocytes; sex-linked recessive disorder.

Chemwatch
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