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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.

ORPHA:329284

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: -

  A summary on this disease is available in Español (2016) Italiano (2016) Nederlands (2016)

Detailed information

General public

Guidelines

Disease review articles

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.