Sällsynt leukoencefalopati med anhoppning av Rosenthalfibrer i subpiala, periventrikulära och subependymala hjärnzoner. Oftast debuterar i spädbarnsåldern. Sjukdomen som debuterar i undomsåldern eller vuxenåldern visar progressiv atrofi i nedre hjärnstammen. De novo GFAP mutationer visar benägenhet för paternal nedärvning.
Rare leukoencephalopathy with infantile-onset accumulation of Rosenthal fibers in the subpial, periventricular, and subependymal zones of the brain. Rosenthal fibers are GLIAL FIBRILLARY ACIDIC PROTEIN aggregates found in ASTROCYTES. Juvenile- and adult-onset types show progressive atrophy of the lower brainstem instead. De novo mutations in the GFAP gene are associated with the disease with propensity for paternal inheritance.
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Demyelinogenic Leukodystrophy — Fibrinoid Degeneration of Astrocytes — Leukodystrophy with Rosenthal Fibers — Alexander's Disease — Alexanders Disease — Dysmyelinogenic Leukodystrophy