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Neurodegeneration with brain iron accumulation

Disease definition

Neurodegeneration with brain iron accumulation (NBIA, formerly Hallervorden-Spatz syndrome) encompasses a group of rare neurodegenerative disorders characterized by progressive extrapyramidal dysfunction (dystonia, rigidity, choreoathetosis), iron accumulation in the brain and the presence of axonal spheroids, usually limited to the central nervous system.


  • Synonym(s):
    • NBIA
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal dominant or Autosomal recessive or X-linked dominant 
  • Age of onset: Adult, Adolescent, Childhood, Infancy
  • ICD-10: G23.0
  • OMIM: -
  • UMLS: C2931845
  • MeSH: C538421
  • GARD: 11899
  • MedDRA: -

Detailed information

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