RARE DISEASERESEARCH ATLAS
Open navigation menu

ORPHA:100070

Progressive non-fluent aphasia

high confidenceDisorder

Also known as: Agramatic variant of PPA · Agramatic variant of primary progressive aphasia · Non-fluent variant PPA

Publications

1,189

91.6th percentile

Trials

6

Interventional, condition-specific

Researchers

1,267

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

non-fluent aphasia (PNFA) is a form of frontotemporal dementia (FTD), characterized by agrammatism, laborious speech, alexia, and agraphia, frequently accompanied by apraxia of speech (AOS). Language comprehension is relatively preserved.

How rare: 1-9 / 100 000 — about one to nine people per hundred thousand.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (2)

Primary Progressive Nonfluent aphasia · non-fluent variant PPA

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

2/6 stages with a signal

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    1,189 matched papers (634 in last 10 years) Source

  3. Phenotype characterisedNot checked

    Not yet enriched from Monarch / HPO

  4. Animal modelNot checked

    Not yet enriched from Monarch / Alliance

  5. Orphan designationNot checked

    FDA/EMA orphan-drug designation not enriched yet

  6. Interventional trialPresent

    6 matched on ClinicalTrials.gov (1 recruiting in sample)

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

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.

Phenotypes (Monarch / HPO)

Not enriched in this build — Monarch phenotype joins were not run for this record.

Animal models (Monarch / Alliance)

Not enriched in this build.

Literature

Is anyone studying this?

1,189

1,189 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=5449) is 41.

1,189 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).

634 in the last 10 years · high confidence · 91.6th percentile (publications denominator)

Phrase hits: 1,189 · MeSH hits: 7

Open Europe PMC search

Who's working on it?

1,267

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.

  1. 01
    Piguet O16 papers · 2026

    The University of Sydney, School of Psychology, Sydney, Australia; The University of Sydney, Brain and Mind Centre, Sydney, Australia.

    Papers in Europe PMC
  2. 02
    Ahmed RM10 papers · 2026

    The University of Sydney, Brain and Mind Centre, Sydney, Australia; Royal Prince Alfred Hospital, Sydney, Australia.

    Papers in Europe PMC
  3. 03
    Hodges JR8 papers · 2021

    The University of Sydney, Brain and Mind Centre, Sydney, Australia.

    Papers in Europe PMC
  4. 04
    Burrell JR7 papers · 2025

    Prince of Wales Medical Research Institute, University of New South Wales, Sydney, Australia.

    Papers in Europe PMC
  5. 05
    Irish M7 papers · 2026

    The University of Sydney, School of Psychology, Sydney, Australia; The University of Sydney, Brain and Mind Centre, Sydney, Australia. Electronic address: muireann.irish@sydney.edu.au.

    Papers in Europe PMC
  6. 06
    Foxe D6 papers · 2025

    Brain and Mind Centre, The University of Sydney, Sydney, NSW 2050, Australia.

    Papers in Europe PMC
  7. 07
    Warren JD6 papers · 2025

    Dementia Research Centre, UCL Queen Square Institute of Neurology, University College London, London, United Kingdom.

    Papers in Europe PMC
  8. 08
    Kumfor F5 papers · 2025

    The University of Sydney, Brain & Mind Centre, Sydney, NSW, Australia; The University of Sydney, School of Psychology, Sydney, NSW, Australia. Electronic address: fiona.kumfor@sydney.edu.au.

    Papers in Europe PMC
  9. 09
    Rohrer JD5 papers · 2025

    Dementia Research Centre, UCL Queen Square Institute of Neurology, University College London, London, United Kingdom.

    Papers in Europe PMC
  10. 10
    Thompson JC5 papers · 2025

    Manchester Academic Health Sciences Centre, Cerebral Function Unit, Greater Manchester Neuroscience Centre, Salford Royal NHS Foundation Trust, Salford, Manchester, UK; Institute of Brain, Behaviour and Mental Health, University of Manchester, Manchester, UK.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

6

interventional trials for this specific condition

6 interventional trials matched this specific condition name; 1 currently recruiting in our sample.

Data as of 27 July 2026

6 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 89th percentile).

high confidence · 89th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

6 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

Open the complete matched search on ClinicalTrials.gov

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 2026-07-26

This 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 on the overview but are not added to the query string.

"Progressive non-fluent aphasia" OR "Agramatic variant of PPA" OR "Agramatic variant of the PPA" OR "Agramatic variant of primary progressive aphasia" OR "Agramatic variant of the primary progressive aphasia" OR "Non-fluent variant PPA" OR "Primary Progressive Nonfluent aphasia"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Primary Progressive Nonfluent Aphasia

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Progressive non-fluent aphasia" OR "Agramatic variant of PPA" OR "Agramatic variant of the PPA" OR "Agramatic variant of primary progressive aphasia" OR "Agramatic variant of the primary progressive aphasia" OR "Non-fluent variant PPA" OR "Primary Progressive Nonfluent aphasia"

Interventional trials matched via: both, 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: 6 interventional · 1 observational · 0 expanded access. Only interventional studies enter the trial headline.

Query health: ok — strategies attempted: phrase, mesh; with hits: phrase, mesh

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

Ingested 2026-07-27T06:59:38.767Z