Alexander Disease

**Definition:** 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.

**Tree numbers:** - C10.228.140.163.100.362.312 - C10.228.140.695.625.312 - C10.314.400.312 - C10.574.500.024 - C16.320.400.024 - C16.320.565.189.362.312 - C18.452.132.100.362.312 - C18.452.648.189.362.312

**Synonyms:** - Alexander's Disease - Demyelinogenic Leukodystrophy - Dysmyelinogenic Leukodystrophy - Fibrinoid Degeneration of Astrocytes - Leukodystrophy with Rosenthal Fibers