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idiopathic hemiconvulsion-hemiplegia syndrome - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

idiopathic hemiconvulsion-hemiplegia syndrome

MONDO:0019485

Also known as:HHE syndromeIHHShemiconvulsion-hemiplegia-epilepsy syndrome

Overview

A rare acute encephalopathy with inflammation-mediated status epilepticus characterized by infancy-onset of refractory unilateral, mainly clonic status epilepticus during or shortly after a febrile episode without evidence of central nervous system infection, followed by permanent or transient hemiplegia with a minimum duration of one week. The majority of children develop pharmaco-resistant epilepsy a few months later. Brain imaging shows edematous swelling of the affected hemisphere at the time of the initial status, followed by hemiatrophy that does not correlate with any vascular territory.

ResearchLimited published research (9 articles)

Diagnosis

Biomarker and diagnostic research for idiopathic hemiconvulsion-hemiplegia syndrome 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.

No clinical trials have been registered for idiopathic hemiconvulsion-hemiplegia syndrome.

9 publications have been identified in PubMed for idiopathic hemiconvulsion-hemiplegia syndrome. Research spans Case Report / Case Series (56%), Diagnostic / Biomarker (22%), and Review / Meta-Analysis (11%).

Recent Publications

S Couto RA (2026). [PMID: 41484385](https://pubmed.ncbi.nlm.nih.gov/41484385/). *Pediatric radiology*. [Case Report / Case Series]
Ciemniejewski B (2026). [PMID: 41661414](https://pubmed.ncbi.nlm.nih.gov/41661414/). *Neurologia i neurochirurgia polska*. [Case Report / Case Series]
Wohlgemuth C (2026). [PMID: 41558656](https://pubmed.ncbi.nlm.nih.gov/41558656/). *Neuropediatrics*. [Case Report / Case Series]
Maeda K (2025). [PMID: 39628036](https://pubmed.ncbi.nlm.nih.gov/39628036/). *Epilepsia open*. [Diagnostic / Biomarker]
Paudel S (2025). [PMID: 40225187](https://pubmed.ncbi.nlm.nih.gov/40225187/). *Case reports in radiology*. [Case Report / Case Series]

Data assembled from 3 of 12 sources · Last updated Sep 20, 2026, 9:53 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:86908

GARD

Genetic and Rare Diseases Info Center

GARD:19085

Questions for Your Care Team

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idiopathic hemiconvulsion-hemiplegia syndrome

Overview

Diagnosis

Research Landscape

Recent Publications

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team