Alexander Disease
MESHD038261
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.
Synonyms
- Alexander's Disease
- Alexanders Disease
- Demyelinogenic Leukodystrophy
- Dysmyelinogenic Leukodystrophy
- Fibrinoid Degeneration of Astrocytes
- Leukodystrophy with Rosenthal Fibers
Source last updated 2026-05-24T15:25:17.568Z