Search for a rare disease

* (*) mandatory field

Other search option(s)

Suggest an update

(*) Required fields.


Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

Captcha image

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.


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
  • UMLS: C0393590
  • MeSH: -
  • GARD: 6406
  • MedDRA: 10059626

Detailed information


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.