Orphanet: Neurodegeneratie geassocieerd met b�ta propellerprote�ne

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Beta-propeller protein-associated neurodegeneration

Disease definition

Beta-propeller protein-associated neurodegeneration (BPAN), also known as static encephalopathy of childhood with neurodegeneration in adulthood, is a rare form of neurodegeneration with brain iron accumulation (NBIA) characterized by early-onset developmental delay and further neurological deterioration in early adulthood.


Classification level: Disorder
  • Synonym(s):
    • BPAN
    • NBIA5
    • Neurodegeneration with brain iron accumulation type 5
    • SENDA
    • Static encephalopathy of childhood with neurodegeneration in adulthood
  • Prevalence: <1 / 1 000 000
  • Inheritance: -
  • Age of onset: Childhood
  • ICD-10: G23.0
  • OMIM: 300894
  • UMLS: -
  • MeSH: -
  • GARD: 12570
  • MedDRA: -

Detailed information

Article for general public


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