ORPHA:275864
Behavioral variant of frontotemporal dementia
Also known as: bv-FTD
Query health: suspect — Only one of 2 strategies returned hits (phrase).
Clinical definition (Orphanet)
Behavioral variant of frontotemporal dementia (bv-FTD) is a form of frontotemporal dementia (FTD), characterized by behavioral impairment and a decline in executive function with frontal lobe-predominant atrophy.
How rare: 1-9 / 100 000 — about one to nine people per hundred thousand.
Is anyone studying this?
1,137
1,137 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.
1,137 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).
820 in the last 10 years · high confidence · 93.2th percentile (publications denominator)
Is a treatment being tested?
4
trials for this specific condition
4 interventional trials matched this specific condition name; 1 currently recruiting in our sample. 98 trials are registered for frontotemporal dementia, the broader category — shown separately because they may or may not enrol this specific subtype.
Data as of 26 July 2026
98
trials for frontotemporal dementia, the broader category this belongs to
Trials registered for a broader category may or may not enrol people with this specific subtype — eligibility criteria vary, and the trial record often doesn't say. Worth raising with a clinician. How we count trials.
4 interventional trials — more than 59.2% of diseases in the trials denominator have none at all (151 of 255; this disease is at the 81.4th percentile).
high confidence · 81.4th 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?
1,358
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.
- 01Miller BL11 papers · 2026
Memory and Aging Center, University of California Memory and Aging Center, San Francisco, California, USA.
Papers in Europe PMC - 02Borroni B8 papers · 2026
Department of Clinical and Experimental Sciences, , Viale Europa, 11 , ,
Papers in Europe PMC - 03Ducharme S8 papers · 2025
McConnell Brain Imaging Centre, Montreal Neurological Institute, McGill University, Montreal, Quebec, QC, Canada.
Papers in Europe PMC - 04Galimberti D8 papers · 2025
Neurodegenerative Disease Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Papers in Europe PMC - 05Otto M8 papers · 2025
Department of Neurology, University Clinic, Martin Luther University Halle-Wittenberg, Halle (Saale), Germany.
Papers in Europe PMC - 06Seeley WW7 papers · 2026
Memory and Aging Center, Department of Neurology, University of California, San Francisco, San Francisco.
Papers in Europe PMC - 07Gorno-Tempini ML6 papers · 2026
Neurology, University of California Memory and Aging Center, San Francisco, California, USA.
Papers in Europe PMC - 08Pijnenburg YAL6 papers · 2025
Alzheimer Center Amsterdam, Department of Neurology, Amsterdam Neuroscience, Vrije Universiteit Amsterdam, Amsterdam UMC, Amsterdam, The Netherlands.
Papers in Europe PMC - 09Rabinovici GD6 papers · 2026
Memory and Aging Center, Department of Neurology, University of California, San Francisco, San Francisco.
Papers in Europe PMC - 10Rowe JB6 papers · 2025
MRC Cognition and Brain Sciences Unit, University of Cambridge, Cambridge, United Kingdom; Department of Clinical Neurosciences and Cambridge University Hospitals NHS Trust, University of Cambridge, United Kingdom. Electronic address: james.rowe@mrc-cbu.cam.ac.uk.
Papers in Europe PMC
Recruiting interventional trials
Trials testing a treatment from the matched ClinicalTrials.gov set
4 interventional trials matched after quoted-phrase search and title/condition post-filter.
Observational and natural-history studies
1 observational study matches this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.
Recruiting or not-yet-recruiting
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.
"Behavioral variant of frontotemporal dementia" OR "bv-FTD"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Behavioral variant of frontotemporal dementia" OR "Behavioral variant of the frontotemporal dementia" OR "bv-FTD" OR "hereditary dementia"
Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).
Study-type breakdown: 4 interventional · 1 observational · 0 expanded access. Only interventional studies enter the trial headline.
Cross-references (from Mondo): UMLS:C4011788
Query health: suspect — strategies attempted: phrase, recall-expansion; with hits: phrase
Run this search on ClinicalTrials.gov
Confidence reasoning
- Preferred label is multi-word and distinctive
- No synonyms dropped by stoplist
- No label/synonym collisions with other diseases in this corpus