Alexander Disease
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.
Year introduced: 2003
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Subheadings:
Tree Number(s): 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
MeSH Unique ID: D038261
Entry Terms:
- Alexander's Disease
- Alexanders Disease
- Demyelinogenic Leukodystrophy
- Dysmyelinogenic Leukodystrophy
- Fibrinoid Degeneration of Astrocytes
- Leukodystrophy with Rosenthal Fibers
See Also: