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Bilateral striopallidodentate calcinosis

^ http://www.orpha.net/ORDO/Orphanet_1980


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.

Synonyms: PFBC, Primary familial brain calcification, BSPDC, Idiopathic basal ganglia calcification, Cerebrovascular ferrocalcinosis

Term info

database cross reference
  • OMIM:616413 (BTNT (broader term maps to a narrower term))
  • MedDRA:10059626 (E (exact mapping (the terms and the concepts are equivalent)))
  • ICD-10:G23.8 (Inclusion term (The term is included under a ICD10 category and has not its own code))
  • UMLS:C0393590 (E (exact mapping (the terms and the concepts are equivalent)))
  • OMIM:606656 (BTNT (broader term maps to a narrower term))
  • ICD-10:G23.8 (NTBT (narrower term maps to a broader term))
  • OMIM:615007 (BTNT (broader term maps to a narrower term))
  • OMIM:213600 (E (exact mapping (the terms and the concepts are equivalent)))
  • OMIM:615483 (BTNT (broader term maps to a narrower term))
hasDbXref

ICD-10:G23.8, OMIM:616413, MedDRA:10059626, OMIM:213600, UMLS:C0393590, OMIM:606656, OMIM:615007, OMIM:615483

notation

ORPHA:1980