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mesial temporal lobe epilepsy with hippocampal sclerosis - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

mesial temporal lobe epilepsy with hippocampal sclerosis

MONDO:0020476

Also known as:MTLE-HS

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Overview

Mesial temporal lobe epilepsy with hippocampal sclerosis is a rare epilepsy syndrome defined by seizures 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 asymmetric. It is frequently associated with an initial precipitating event, such as febrile seizures, hypoxia, intracranial infection or head trauma, most often occurring in the first five years of life, followed by a latent period without seizures. Typical seizures consist of a characteristic aura that is frequently a rising epigastric sensation associated with emotional disturbances, illusions, and autonomic symptoms (widened pupils, palpitations), progressive impairment of consciousness, oro-alimentary automatisms (lip smacking, chewing, licking, tooth grinding), behavioral arrest, head deviation, dystonic postures, hand and verbal automatisms. Seizures are followed by postictal dysfunction. Initially, seizures are easily controlled with antiepileptic drugs, later they frequently become refractory and associated with progressive behavioral changes and memory deficits.

Clinical Trials1 registered

Diagnosis

Biomarker and diagnostic research for mesial temporal lobe epilepsy with hippocampal sclerosis has been reported in the published literature.

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

1 clinical trial registered. Interventions under study include procedural interventions. Pipeline includes 1 NA. Research is primarily sponsored by academic and government institutions.

39 publications have been identified in PubMed for mesial temporal lobe epilepsy with hippocampal sclerosis. Research spans Basic Science / Preclinical (31%), Diagnostic / Biomarker (23%), and Clinical Trial Publication (21%).

Research Type	Count	% of Total
Laboratory research	12

Data assembled from 4 of 12 sources · Last updated Sep 20, 2026, 1:01 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:99701

GARD

Genetic and Rare Diseases Info Center

GARD:19684

Questions for Your Care Team

→Are any of the 1 active clinical trial a good fit for my situation?
→Are there specialists or centers of excellence for this condition in my area?
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mesial temporal lobe epilepsy with hippocampal sclerosis

Overview

Diagnosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

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