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Bilateral striopallidodentate calcinosis

Disease definition

Bilateral striopallidodentate calcinosis (BSPDC, also erroneously called Fahr disease) is characterized by the accumulation of calcium deposits in different brain regions, particularly the basal ganglia and dentate nucleus, and is often associated with neurodegeneration.

ORPHA:1980

  • Synonym(s):
    • BSPDC
    • Cerebrovascular ferrocalcinosis
    • Idiopathic basal ganglia calcification
    • PFBC
    • Primary familial brain calcification
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant or Not applicable 
  • Age of onset: Adult
  • ICD-10: G23.8
  • OMIM: 213600  606656  615007  615483  616413
  • UMLS: C0393590
  • MeSH: -
  • GARD: 6406
  • MedDRA: 10059626

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