ORPHA:99701
Mesial temporal lobe epilepsy with hippocampal sclerosis
Also known as: HS-MTLE · Hippocampal sclerosis-related mesial temporal lobe epilepsy · MTLE-HS
Publications
752
89.4th percentile
Trials
1
Interventional, condition-specific
Researchers
1,369
Distinct authors in sample
Gene link
—
Readiness
2/6
Stages with a signal
Clinical definition (Orphanet)
Mesial temporal lobe with hippocampal sclerosis is a rare syndrome defined by originating in limbic areas of the mesial temporal lobe, particularly in the hippocampus, amygdala, and in the parahippocampal gyrus and its connections, and hippocampal sclerosis, usually unilateral or assymetric. It is frequently associated with an initial precipitating event, such as febrile , hypoxia, intracranial infection or head trauma, most often occurring in the first five years of life, followed by a latent period without . Typical consist of a characteristic aura that is frequently a rising epigastric sensation associated with emotional disturbances, illusions, and autonomic symptoms (widened pupils, palpitations), impairment of consciousness, oro-alimentary automatisms (lip smacking, chewing, licking, tooth grinding), behavioral arrest, head deviation, dystonic postures, hand and verbal automatisms. are followed by postictal dysfunction. Initially, are easily controlled with antiepileptic drugs, later they frequently become refractory and associated with behavioral changes and memory deficits.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0020476
- UMLS:C4749367
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.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
752 matched papers (460 in last 10 years) Source
- Phenotype characterisedNot checked
Not yet enriched from Monarch / HPO
- Animal modelNot checked
Not yet enriched from Monarch / Alliance
- Orphan designationNot checked
FDA/EMA orphan-drug designation not enriched yet
- Interventional trialPresent
1 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?
752
752 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.
752 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).
460 in the last 10 years · high confidence · 89.4th percentile (publications denominator)
Phrase hits: 752 · MeSH hits: 0
Who's working on it?
1,369
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.
- 01Chaves J12 papers · 2026
Serviço de Neurologia, Hospital de Santo António, Centro Hospitalar e Universitário do Porto, Largo Prof. Abel Salazar, 4099-003 Porto, Portugal.
Papers in Europe PMC - 02Banerjee J9 papers · 2026
Centre of Excellence for Epilepsy, AIIMS, New Delhi, India.
Papers in Europe PMC - 03Baykan B9 papers · 2022
Department of Neurology, İstanbul University İstanbul School of Medicine, İstanbul, Turkey.
Papers in Europe PMC - 04Leal B9 papers · 2026
UMIB - Instituto de Ciências Biomédicas Abel Salazar [ICBAS], Universidade do Porto, Rua Jorge Viterbo Ferreira, 228, 4050-313 Porto, Portugal; Lab. Imunogenética, DPIM, ICBAS-UPorto, Rua Jorge Viterbo Ferreira, 228, 4050-313 Porto, Portugal. Electronic address: baleal@icbas.up.pt.
Papers in Europe PMC - 05Lopes J9 papers · 2026
Serviço de Neurofisiologia, Hospital de Santo António, Centro Hospitalar do Porto, Largo Prof. Abel Salazar, 4099-003 Porto, Portugal.
Papers in Europe PMC - 06
- 07Tripathi M9 papers · 2026
Centre of Excellence for Epilepsy, AIIMS, New Delhi, India.
Papers in Europe PMC - 08Zhang J9 papers · 2026
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Papers in Europe PMC - 09Chandra PS8 papers · 2026
Centre of Excellence for Epilepsy, AIIMS, New Delhi, India.
Papers in Europe PMC - 10Ramalheira J8 papers · 2024
Serviço de Neurofisiologia, Hospital de Santo António, Centro Hospitalar do Porto, Largo Prof. Abel Salazar, 4099-003 Porto, Portugal.
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; 1 currently recruiting in our sample.
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).
high 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.
- NCT06558864·NOT YET RECRUITING·High Selective Subiculum SEEG Guided RF-TC for mTLE-HS
Conditions: Mesial Temporal Lobe Epilepsy With Hippocampal Sclerosis · Epilepsy·Matched via name phrase
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 on the overview but are not added to the query string.
"Mesial temporal lobe epilepsy with hippocampal sclerosis" OR "HS-MTLE" OR "Hippocampal sclerosis-related mesial temporal lobe epilepsy" OR "MTLE-HS"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Mesial temporal lobe epilepsy with hippocampal sclerosis" OR "HS-MTLE" OR "Hippocampal sclerosis-related mesial temporal lobe epilepsy" OR "MTLE-HS"
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 · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.
Query health: ok — strategies attempted: phrase; 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
Ingested 2026-07-27T06:12:47.548Z