Alexander Disease

Synonyms

Alexander's Disease

Alexanders Disease

Demyelinogenic Leukodystrophy

Dysmyelinogenic Leukodystrophy

Fibrinoid Degeneration of Astrocytes

Leukodystrophy with Rosenthal Fibers

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.