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ORPHA:238624

Idiopathic intracranial hypertension

medium confidenceDisorder

Also known as: Benign intracranial hypertension · IIH · Pseudotumor cerebri

Publications

12,386

98th percentile

Trials

37

Interventional, condition-specific

Researchers

1,167

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

intracranial hypertension is a neurological disorder characterized by isolated increased intracranial pressure manifesting with recurrent and persistent headaches, nausea, vomiting, and transient obstruction of the visual field, papilledema. Visual loss can be irreversible.

How rare: 1-5 / 10 000 — about one to five people per ten thousand (still uncommon, but less ultra-rare).

Orphanet entry

Cross-references

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

Additional Mondo synonyms (5)

Idiopathic Intracranial Hypertension · benign intracran. hypt. · benign intracranial hypertension · idiopathic intracranial hypertension · pseudotumor cerebri

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

    12,386 matched papers (6,426 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

    37 matched on ClinicalTrials.gov (10 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?

12,386

12,386 papers — among the better-studied rare conditions, though still a fraction of common-disease literature (breast cancer: over 700,000). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.

12,386 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).

6,426 in the last 10 years · medium confidence · 98th percentile (publications denominator)

Phrase hits: 12,386 · MeSH hits: 125

Open Europe PMC search

Who's working on it?

1,167

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
    Mollan SP6 papers · 2026

    Metabolism and Systems Science, School of Medical Sciences, College of Medicine and Health, University of Birmingham, Birmingham, UK.

    Papers in Europe PMC
  2. 02
    Hansen NS5 papers · 2026

    Department of Neurology, Danish Headache Center, Copenhagen University Hospital - Rigshospitalet, Glostrup, Denmark.

    Papers in Europe PMC
  3. 03
    Jensen RH5 papers · 2026

    Department of Neurology, Danish Headache Center, Copenhagen University Hospital - Rigshospitalet, Glostrup, Denmark.

    Papers in Europe PMC
  4. 04
    Beier D4 papers · 2026

    Department of Neurology, Odense University Hospital, Odense, Denmark.

    Papers in Europe PMC
  5. 05
    Bender MT4 papers · 2026

    Department of Neurosurgery, University of Rochester Medical Center, Rochester, NY, USA.

    Papers in Europe PMC
  6. 06
    Bsteh G4 papers · 2026

    Department of Neurology, Medical University of Vienna, Waehringer Guertel 18-20, Vienna, 1090, Austria. gabriel.bsteh@meduniwien.ac.at.

    Papers in Europe PMC
  7. 07
    Davies JM4 papers · 2026

    Department of Neurosurgery, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo , New York , USA.

    Papers in Europe PMC
  8. 08
    Fargen KM4 papers · 2025

    Neurological Surgery and Radiology, Wake Forest University, Winston-Salem, North Carolina, USA.

    Papers in Europe PMC
  9. 09
    Grech O4 papers · 2026

    Metabolism and Systems Science, School of Medical Sciences, College of Medicine and Health, University of Birmingham, Birmingham, UK.

    Papers in Europe PMC
  10. 10
    Pemp B4 papers · 2026

    Department of Ophthalmology, Medical University of Vienna, Vienna, Austria.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

37

interventional trials for this specific condition

37 interventional trials matched this specific condition name; 10 currently recruiting in our sample. 44 trials are registered for intracranial hypertension, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 27 July 2026

37 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 96.3th percentile).

medium confidence · 96.3th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

37 interventional trials matched after quoted-phrase search and title/condition post-filter.

Broader category: intracranial hypertension

44

Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.

Worth raising with a clinician. How we count trials.

Recruiting under the broader category

Observational and natural-history studies

16 observational studies match 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.

"Idiopathic intracranial hypertension" OR "Benign intracranial hypertension" OR "Pseudotumor cerebri" OR "benign intracran. hypt."

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Pseudotumor Cerebri

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

"Idiopathic intracranial hypertension" OR "Benign intracranial hypertension" OR "Pseudotumor cerebri" OR "benign intracran. hypt."

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: 37 interventional · 16 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"intracranial hypertension"

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

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: IIH

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

Ingested 2026-07-27T10:25:12.940Z