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ORPHA:1930

Herpes simplex virus encephalitis

medium confidenceDisorder

Also known as: HSE · HSV encephalitis · HSVE · Herpes simplex meningo-encephalitis · Herpes simplex neuroinvasion · Herpetic encephalitis

Query health: suspect — Only one of 2 strategies returned hits (phrase).

Publications

5,227

95.7th percentile

Trials

1

Interventional, condition-specific

Researchers

1,073

Distinct authors in sample

Gene link

GTF3A

Limited

Readiness

3/6

Stages with a signal

Clinical definition (Orphanet)

A rare disorder caused by infection of the central nervous system by Herpes simplex virus (HSV) that could have a devastating clinical course and a potentially fatal outcome particularly with delay or lack of treatment. This disorder often involves the frontal and temporal lobes, usually asymmetrically, resulting in personality changes, cognitive impairment, aphasia, , and focal weakness.

How rare: 1-9 / 1 000 000 — roughly one to nine people per million. In a city the size of Kolkata, perhaps a few dozen.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (4)

Encephalitis, Herpes Simplex · Simplexvirus caused infectious encephalitis · Simplexvirus infectious encephalitis · herpetic encephalitis

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.”

3/6 stages with a signal

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

  1. Gene identifiedPresent

    Limited — GTF3A

  2. LiteraturePresent

    5,227 matched papers (2,266 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

    1 matched on ClinicalTrials.gov

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?

Possibly — only limited evidence so far for GTF3A.

GenCC classification: Limited.

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?

5,227

5,227 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.

5,227 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).

2,266 in the last 10 years · medium confidence · 95.7th percentile (publications denominator)

Phrase hits: 5,227 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,073

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
    Michael BD8 papers · 2026

    Liverpool Interdisciplinary Neuroscience Centre (LINC), University of Liverpool, Liverpool, L9 7AL, UK.

    Papers in Europe PMC
  2. 02
    Easton A5 papers · 2026

    Clinical Infection, Microbiology & Immunology, Institute of Infection, Veterinary and Ecological Sciences, University of Liverpool, Liverpool, CH64 7TE, UK.

    Papers in Europe PMC
  3. 03
    Ellul MA4 papers · 2026

    Brain Infection and Inflammation Group, Clinical Infection Microbiology and Immunology, Institute of Infection Veterinary and Ecological Sciences, University of Liverpool, Liverpool.

    Papers in Europe PMC
  4. 04
    Solomon T4 papers · 2026

    Institute of Infection, Veterinary and Ecological Sciences, University of Liverpool, 8 West Derby Street, Liverpool, UK

    Papers in Europe PMC
  5. 05
    Cotton F3 papers · 2024

    Service de Radiologie, Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Chemin du Grand-Revoyet, Pierre-Bénite 69495, France; INSA-Lyon, Université Claude Bernard Lyon 1, UJM-Saint Etienne, CNRS, Inserm, CREATIS UMR 5220, U1206, Université de Lyon, Pierre-Bénite F-69495, France. Electronic address: francois.cotton@chu-lyon.fr.

    Papers in Europe PMC
  6. 06
    Defres S3 papers · 2026

    Institute of Infection, Veterinary and Ecological Sciences, University of Liverpool, 8 West Derby Street, Liverpool, UK

    Papers in Europe PMC
  7. 07
    Dumez P3 papers · 2025

    French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hôpital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, 59 Bd Pinel, 69677, Bron cedex, France.

    Papers in Europe PMC
  8. 08
    Honnorat J3 papers · 2025

    French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hôpital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, 59 Bd Pinel, 69677, Bron cedex, France. jerome.honnorat@chu-lyon.fr.

    Papers in Europe PMC
  9. 09
    Joubert B3 papers · 2024

    Centre National de Référence Pour les Syndromes Neurologiques Paranéoplasiques, Hôpital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, Bron, France.

    Papers in Europe PMC
  10. 10
    Kneen R3 papers · 2026

    Institute of Infection, Veterinary and Ecological Sciences, University of Liverpool, 8 West Derby Street, Liverpool, UK

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

1

interventional trials for this specific condition

1 interventional trial matched this specific condition name; none in our sample are currently recruiting.

Data as of 27 July 2026

1 interventional trial — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 76.8th percentile).

medium confidence · 76.8th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

No currently recruiting studies in the matched set. Open the same search on ClinicalTrials.gov.

Observational and natural-history studies

3 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.

None of the matched observational studies is currently listed as 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.

"Herpes simplex virus encephalitis" OR "HSV encephalitis" OR "Herpes simplex meningo-encephalitis" OR "Herpes simplex neuroinvasion" OR "Herpetic encephalitis" OR "Encephalitis, Herpes Simplex" OR "Simplexvirus caused infectious encephalitis" OR "Simplexvirus infectious encephalitis"

Run this search on Europe PMC

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

"Herpes simplex virus encephalitis" OR "HSV encephalitis" OR "Herpes simplex meningo-encephalitis" OR "Herpes simplex neuroinvasion" OR "Herpetic encephalitis" OR "Encephalitis, Herpes Simplex" OR "Simplexvirus caused infectious encephalitis" OR "Simplexvirus infectious encephalitis" OR "GTF3A"

Recall-expansion terms: GTF3A

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: 1 interventional · 3 observational · 0 expanded access. Only interventional studies enter the trial headline.

Query health: suspect — strategies attempted: phrase, recall-expansion; with hits: phrase

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: HSE; HSVE

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • 2 synonym(s) dropped by stoplist (may under-count)
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-26T18:32:28.147Z