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early-infantile DEE - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

early-infantile DEE

MONDO:0800491

Also known as:epileptic seizures - myoclonicepileptic seizures, myoclonicmyoclonia epileptica

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Overview

A neonatal/infantile epilepsy syndrome characterized by frequent drug-resistant seizures that begin ≤3 months of age, with abnormal interictal EEG and neurological examination. In up to 80% of patients, EIDEE is caused by an underlying structural, genetic, or metabolic reason.

OnsetINFANTILE

TreatmentsNo approved treatments · 1 in pipeline

Clinical Trials2 registered · 2 recruiting

Diagnosis

Biomarker and diagnostic research for early-infantile DEE has been reported in the published literature.

Treatment

Compiled from FDA-approved drug records, orphan drug designations, and FDA Adverse Event Reporting System (FAERS) safety data. GeneReviews management guidance is included where available.

No approved treatments are currently available for early-infantile DEE. An additional 1 compound holds orphan drug designation.

Orphan Drug Designations

While no drugs are FDA-approved specifically for early-infantile DEE, some of the following designated compounds may be used off-label in clinical practice. Treatment decisions should be made in consultation with a specialist familiar with this condition.

The following drugs have received orphan drug designation from the FDA for early-infantile DEE. Orphan designation reflects regulatory interest and does not indicate approval for treatment.

Brand Name	Generic Name	Sponsor

Prognosis

Estimated prevalence: Unknown (Unknown prevalence).

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.

2 clinical trials registered, 2 recruiting. Interventions under study include other interventions. Research is primarily sponsored by academic and government institutions.

182 publications have been identified in PubMed for early-infantile DEE. Research spans Review / Meta-Analysis (32%), Epidemiology / Natural History (19%), and Basic Science / Preclinical (16%).

Research Type	Count	% of Total
Research summaries	55	32%

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 1:58 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:1934 Orphanet:1935

Questions for Your Care Team

→Are any of the 2 active clinical trials a good fit for my situation?
→What is the current development status of Clemizole Hydrochloride, and when might it become available?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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early-infantile DEE

Overview

Diagnosis

Treatment

Orphan Drug Designations

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

Subtypes

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Clinical Trials

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