Alexander Disease
Also known asAlexander's Disease · Alexanders Disease · Demyelinogenic Leukodystrophy · Dysmyelinogenic Leukodystrophy · Fibrinoid Degeneration of Astrocytes · Leukodystrophy with Rosenthal Fibers
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.
MeSH classification
- 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
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Source: NIH MeSH 2026 (D038261) — National Library of Medicine, public domain. View official MeSH record ↗