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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

Classification level: Disorder
  • 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  615007  615483  616413  618824
  • UMLS: C0393590
  • MeSH: -
  • GARD: 6406
  • MedDRA: 10059626

Detailed information

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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.