ORPHA:1980
Bilateral striopallidodentate calcinosis
Also known as: BSPDC · Cerebrovascular ferrocalcinosis · Idiopathic basal ganglia calcification · PFBC · Primary familial brain calcification
Clinical definition (Orphanet)
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.
How rare: <1 / 1 000 000 — fewer than one in a million. In a city the size of Kolkata, that might mean on the order of fifteen people.
Is anyone studying this?
2,078
2,078 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=183) is 38.
2,078 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 38 (publications denominator n=183).
1,258 in the last 10 years · low confidence
Is a treatment being tested?
1
trials for this specific condition
1 interventional trial matched this specific condition name; 1 currently recruiting in our sample.
Data as of 26 July 2026
1 interventional trial — more than 59.2% of diseases in the trials denominator have none at all (151 of 255; this disease is at the 65.3th percentile).
low confidence · 65.3th percentile (trials denominator)
Do we know what causes it?
Not yet — the cause hasn't been pinned down in GenCC.
No strong gene–disease assertion joined for this Orphanet entity.
Who's working on it?
1,062
Distinct author names in 200 sampled papers — named people below.
Who's working on it?
People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.
- 01Hozumi I11 papers · 2026
Laboratory of Medical Therapeutics and Molecular Therapeutics, Gifu Pharmaceutical University, Gifu, Japan. Electronic address: hozumi@gifu-pu.ac.jp.
Papers in Europe PMC - 02Luo W9 papers · 2026
Department of Neurology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
Papers in Europe PMC - 03Kurita H8 papers · 2025
Laboratory of Medical Therapeutics and Molecular Therapeutics, Gifu Pharmaceutical University, Gifu, Japan.
Papers in Europe PMC - 04Cen Z7 papers · 2026
Department of Neurology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
Papers in Europe PMC - 05Li J7 papers · 2026
Department of Neurosurgery, Xiangya Hospital, Central South University, Changsha, China.
Papers in Europe PMC - 06Yang D7 papers · 2026
Department of Neurology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
Papers in Europe PMC - 07Chen Y6 papers · 2025
The First School of Medicine, School of Information and Engineering, Wenzhou Medical University, Wenzhou, China.
Papers in Europe PMC - 08Inden M6 papers · 2025
Laboratory of Medical Therapeutics and Molecular Therapeutics, Gifu Pharmaceutical University, Gifu, Japan.
Papers in Europe PMC - 09Nicolas G6 papers · 2026
Univ Rouen Normandie, Inserm U1245 and CHU Rouen, Department of Genetics, CNRMAJ and Reference Center for Neurogenetics Disorders, F-76000, Rouen, France. gaelnicolas@hotmail.com.
Papers in Europe PMC - 10Wang H6 papers · 2026
Department of Neurology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
Papers in Europe PMC
Recruiting interventional trials
Trials testing a treatment from the matched ClinicalTrials.gov set
1 interventional trials matched after quoted-phrase search and title/condition post-filter.
Where to find support
We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:
India — NPRD
Last verified 2026-07-26This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.
Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.
How we counted this
Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored below but are not added to the query string.
"Bilateral striopallidodentate calcinosis" OR "BSPDC" OR "Cerebrovascular ferrocalcinosis" OR "Idiopathic basal ganglia calcification" OR "Primary familial brain calcification" OR "basal ganglia calcification" OR "basal ganglia degeneration with calcification"
MeSH descriptor terms unioned into the query: Fahr's disease
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Bilateral striopallidodentate calcinosis" OR "BSPDC" OR "Cerebrovascular ferrocalcinosis" OR "Idiopathic basal ganglia calcification" OR "Primary familial brain calcification" OR "basal ganglia calcification" OR "basal ganglia degeneration with calcification" OR "Fahr's disease"
Interventional trials matched via: both (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).
Study-type breakdown: 1 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.
Cross-references (from Mondo): MESH:C536275
Query health: ok — strategies attempted: phrase, mesh; with hits: phrase, mesh
Run this search on ClinicalTrials.gov
Synonyms dropped by stoplist: PFBC
Confidence reasoning
- Preferred label is multi-word and distinctive
- 1 synonym(s) dropped by stoplist (may under-count)
- No label/synonym collisions with other diseases in this corpus
- Publication count (2078) is implausibly high for prevalence class "<1 / 1 000 000" — treat as possible over-matching, not a measure of research intensity