Orphanet: Alexander disease

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Alexander disease

Disease definition

A rare neurodegenerative disorder of the astrocytes comprised of two clinical forms: Alexander disease (AxD) type I and type II manifesting with various degrees of macrocephaly, spasticity, ataxia and seizures and leading to psychomotor regression and death.


Classification level: Disorder
  • Synonym(s):
    • AxD
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant 
  • Age of onset: All ages
  • ICD-10: E75.2
  • OMIM: 203450
  • UMLS: C0270726
  • MeSH: D038261
  • GARD: 5774
  • MedDRA: -

Detailed information

Article for general public


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