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Alexander Disease MeSH Descriptor Data 2021


MeSH Heading
Alexander Disease
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
Unique ID
D038261
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/D038261
Scope Note
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.
Entry Term(s)
Alexander's Disease
Demyelinogenic Leukodystrophy
Dysmyelinogenic Leukodystrophy
Fibrinoid Degeneration of Astrocytes
Leukodystrophy with Rosenthal Fibers
See Also
Glial Fibrillary Acidic Protein
Public MeSH Note
2003
History Note
2003
Date Established
2003/01/01
Date of Entry
2002/07/02
Revision Date
2013/07/08
Alexander Disease Preferred
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