JSON

bilateral striopallidodentate calcinosis

^ http://purl.obolibrary.org/obo/MONDO_0008947


A basal ganglia disease characterized by the accumulation of calcium deposits in different brain regions, particularly the basal ganglia and dentate nucleus, and is often associated with neurodegeneration. [ Orphanet:1980 ]

Term info

database cross reference
  • MedDRA:10059626 (Orphanet:1980/e)
  • OMIMPS:213600 (MONDO:equivalentTo)
  • MESH:C536275 (MONDO:equivalentTo)
  • DOID:0060230 (MONDO:equivalentTo)
  • HP:0002135 (MONDO:otherHierarchy)
  • UMLS:CN852731 (MONDO:equivalentTo)
  • ICD9:333.0 (MONDO:relatedTo)
  • SCTID:230311004 (MONDO:equivalentTo)
  • SCTID:110997000 (MONDO:equivalentTo)
  • ICD9:348.89 (MONDO:relatedTo)
  • Orphanet:1980 (OMIM:213600)
  • UMLS:C0393589 (MONDO:equivalentTo)
Subsets

ordo_disease

A synonym that is historic and discouraged
Fahr disease [ DOID:0060230 ]

abbreviation
BSPDC [ Orphanet:1980 ]

abbreviation
PFBC [ Orphanet:1980 ]

closeMatch

http://identifiers.org/meddra/10059626

comment

Encompasses a range of disorders with different etiology, see https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-016-0693-1

definition

A basal ganglia disease characterized by the accumulation of calcium deposits in different brain regions, particularly the basal ganglia and dentate nucleus, and is often associated with neurodegeneration.

exactMatch

http://identifiers.org/snomedct/230311004, http://identifiers.org/snomedct/110997000, http://purl.obolibrary.org/obo/Orphanet_1980, http://linkedlifedata.com/resource/umls/id/CN852731, http://linkedlifedata.com/resource/umls/id/C0393589, https://omim.org/phenotypicSeries/PS213600, http://identifiers.org/mesh/C536275, http://purl.obolibrary.org/obo/DOID_0060230

has exact synonym

cerebrovascular ferrocalcinosis, PFBC, BSPDC, basal ganglia calcification, basal ganglia degeneration with calcification, primary familial brain calcification

has narrow synonym

idiopathic basal ganglia calcification, Fahr disease

has related synonym

basal ganglia calcification, idiopathic

id

MONDO:0008947

Term relations