Diseases

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.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

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 Record

Classification

Broader headings

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MeSH Record

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MeSH Record

Synonyms

6 entry terms
  • Alexander's Disease
  • Demyelinogenic Leukodystrophy
  • Dysmyelinogenic Leukodystrophy
  • Fibrinoid Degeneration of Astrocytes
  • Leukodystrophy with Rosenthal Fibers
  • Alexanders Disease

MeSH Record

Aspects Covered

34 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Record

History Note

2003

MeSH Hierarchy

Tree Numbers

  1. C10w.228w.140w.163.100.362w.312w
  2. C10w.228w.140w.695w.625w.312w
  3. C10w.314w.400w.312w
  4. C10w.574w.500.024w
  5. C16w.320w.400.024w
  6. C16w.320w.565w.189.362w.312w
  7. C18.452w.132.100.362w.312w
  8. C18.452w.648w.189.362w.312w

AMA Style

References

  1. National Library of Medicine. Alexander Disease. Medical Subject Headings (MeSH). 2026. Unique ID D038261. http://id.nlm.nih.gov/mesh/2026/D038261
  2. Alexander Disease. In: Wikipedia. https://en.wikipedia.org/wiki/Alexander_disease
  3. Alexander Disease. In: Wikidata. https://www.wikidata.org/wiki/Q567820