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leukodystrophy with diffuse Rosenthal fiber formation


Definition: 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.

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.