A Study of EPX-100 (Clemizole Hydrochloride) in Participants With Dravet Syndrome

NCT ID: NCT04462770

Last Updated: 2025-08-28

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Clinical Phase

PHASE3

Total Enrollment

150 participants

Study Classification

INTERVENTIONAL

Study Start Date

2020-09-15

Study Completion Date

2029-05-01

Brief Summary

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This is a multicenter, Phase 3, randomized, double-blind, placebo-controlled study designed to evaluate the efficacy and safety of clemizole hydrochloride (EPX-100) as adjunctive therapy in children and adult participants with Dravet syndrome (DS).

Detailed Description

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This is a global, multicenter, randomized, double-blind, placebo-controlled study to evaluate the safety and efficacy of clemizole hydrochloride as adjunctive therapy in children and adult participants with DS. The study consists of a 4-week Observational Period, a 16-week Double-Blind (DB) Period and an Open-Label Extension (OLE) Period for up to 156 weeks.

Conditions

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Dravet Syndrome

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Patients are randomized 1:1 to clemizole HCl (EPX-100) or placebo.
Primary Study Purpose

TREATMENT

Blinding Strategy

TRIPLE

Participants Caregivers Investigators

Study Groups

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Double-blind clemizole HCl

Participants will receive their first dose of study drug following randomization.

Group Type EXPERIMENTAL

Clemizole HCl

Intervention Type DRUG

Clemizole HCl will be administered as an oral solution.

Placebo

Participants will receive their first dose of study drug following randomization.

Group Type PLACEBO_COMPARATOR

Placebo

Intervention Type DRUG

Placebo will be administered as an oral solution.

Open-label clemizole HCl

Eligible participants who complete the DB Period will have the option to continue in the OLE Period, during which they will receive clemizole HCL for up to 3 years.

Group Type EXPERIMENTAL

Clemizole HCl

Intervention Type DRUG

Clemizole HCl will be administered as an oral solution.

Placebo

Intervention Type DRUG

Placebo will be administered as an oral solution.

Interventions

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Clemizole HCl

Clemizole HCl will be administered as an oral solution.

Intervention Type DRUG

Placebo

Placebo will be administered as an oral solution.

Intervention Type DRUG

Other Intervention Names

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EPX-100

Eligibility Criteria

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Inclusion Criteria

1. Male and female participants 2 years and older at time of consent.
2. Participant or parent/legally authorized representative (LAR) willing and able to provide written informed consent, assent (if applicable) prior to initiation of any study related procedures.
3. Clinical diagnosis of DS. Participants must have seizures which are not completely controlled by AEDs with the following criteria:

* Onset of seizures prior to 18 months of age,
* Normal development at onset,
* History of at least one type of countable motor seizure (CMS),
* Brain MRI without cortical malformation (not including mild atrophy associated with the natural progression of DS),
* Genetic mutation of the SCN1A gene must be documented.

Exclusion Criteria

1. Known sensitivity, allergy, or previous exposure to clemizole HCl.
2. Exposure to any investigational drug or device \<90 days prior to screening or plans to participate in another drug or device trial at any time during the study.
3. Seizures secondary to illicit drug (this includes concomitant use of tetrahydrocannabinol \[THC\] and nonprescription cannabidiol preparations) or alcohol use, infection, neoplasm, demyelinating disease, degenerative neurological disease, or central nervous system disease deemed progressive, metabolic illness, or progressive degenerative disease.
4. Concurrent use of lorcaserin. Note: Prior use of lorcaserin is permitted if at least 30 days have passed since the last dose.
5. Concurrent use of fenfluramine.
6. Epilepsy surgery planned during the study or epilepsy surgery within 6 months prior to Screening.
Minimum Eligible Age

2 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Harmony Biosciences Management, Inc.

INDUSTRY

Sponsor Role collaborator

Epygenix

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Amit Ray, MD

Role: STUDY_DIRECTOR

Harmony Biosciences Management, Inc.

Locations

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Children's Hospital of Los Angeles

Los Angeles, California, United States

Site Status TERMINATED

University of California Irvine

Orange, California, United States

Site Status RECRUITING

UCSF Medical Center

San Francisco, California, United States

Site Status RECRUITING

Yale University School of Medicine

New Haven, Connecticut, United States

Site Status NOT_YET_RECRUITING

The Nemours Foundation

Wilmington, Delaware, United States

Site Status RECRUITING

Rare Disease Research FL

Kissimmee, Florida, United States

Site Status NOT_YET_RECRUITING

Pediatric Neurology and Epilepsy Specialists

Winter Park, Florida, United States

Site Status NOT_YET_RECRUITING

Clinical Integrative Research Center of Atlanta (CIRCA)

Atlanta, Georgia, United States

Site Status NOT_YET_RECRUITING

Ann & Robert H. Lurie Children's Hospital of Chicago

Chicago, Illinois, United States

Site Status WITHDRAWN

Norton Children's Research Institute

Louisville, Kentucky, United States

Site Status NOT_YET_RECRUITING

University of Michigan- Mott Children's Hospital

Ann Arbor, Michigan, United States

Site Status WITHDRAWN

Children's Nebraska

Omaha, Nebraska, United States

Site Status RECRUITING

Northeast Regional Epilepsy Group

Hackensack, New Jersey, United States

Site Status TERMINATED

Neurology Center for Epilepsy and Seizures

Marlboro, New Jersey, United States

Site Status ACTIVE_NOT_RECRUITING

Weill Cornell Medical Center

New York, New York, United States

Site Status RECRUITING

Northwell Health - Lenox Hill Hospital

New York, New York, United States

Site Status NOT_YET_RECRUITING

Duke University Health System

Durham, North Carolina, United States

Site Status NOT_YET_RECRUITING

Wake Forest Baptist Medical Center

Winston-Salem, North Carolina, United States

Site Status RECRUITING

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, United States

Site Status RECRUITING

Cleveland Clinic

Cleveland, Ohio, United States

Site Status RECRUITING

Nationwide Children's Hospital

Columbus, Ohio, United States

Site Status WITHDRAWN

The Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Site Status RECRUITING

Le Bonheur Children's Hospital

Memphis, Tennessee, United States

Site Status TERMINATED

Child Neurology Consultants of Austin

Austin, Texas, United States

Site Status RECRUITING

UT Southwestern/Children's Health

Dallas, Texas, United States

Site Status NOT_YET_RECRUITING

University of Utah

Salt Lake City, Utah, United States

Site Status RECRUITING

Seattle Children's Hospital

Seattle, Washington, United States

Site Status RECRUITING

UBC Children's Hospital Research Institute

Vancouver, British Columbia, Canada

Site Status RECRUITING

Children's Hospital of Eastern Ontario Research Institute Inc.

Ottawa, Ontario, Canada

Site Status RECRUITING

The Hospital for Sick Children

Toronto, Ontario, Canada

Site Status RECRUITING

Toronto Western Hospital, University Health Network

Toronto, Ontario, Canada

Site Status RECRUITING

Tbilisi State Medical University, Givi Zhvania Academic, Clinic of Pediatry

Tbilisi, , Georgia

Site Status RECRUITING

Medi Club Georgia LLC

Tbilisi, , Georgia

Site Status RECRUITING

Institute of Neurology and Neuropsychology LTD

Tbilisi, , Georgia

Site Status RECRUITING

Semmelweis University

Budapest, , Hungary

Site Status RECRUITING

University of Debrecen

Debrecen, , Hungary

Site Status RECRUITING

University Clinical Center in Gdansk, Division of Developmental Neurology

Gdansk, , Poland

Site Status NOT_YET_RECRUITING

Medical Centre Plejady

Krakow, , Poland

Site Status RECRUITING

Institute of Mother and Child

Warsaw, , Poland

Site Status RECRUITING

"Prof. Dr. Al. Obregia" Psychiatry Clinical Hospital

Bucharest, , Romania

Site Status NOT_YET_RECRUITING

Hospital Infantil Universitario Niño Jesús

Madrid, Madrid Provincia, Spain

Site Status RECRUITING

Hospital de la Santa Creu i Sant Pau

Barcelona, , Spain

Site Status RECRUITING

Cardiff and Vale University Health Board

Cardiff, , United Kingdom

Site Status RECRUITING

Alder Hey Children's NHS Foundation Trust

Liverpool, , United Kingdom

Site Status NOT_YET_RECRUITING

Great Ormond Street Hospital For Children

London, , United Kingdom

Site Status RECRUITING

Sheffield Children's Hospital

Sheffield, , United Kingdom

Site Status RECRUITING

Countries

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United States Canada Georgia Hungary Poland Romania Spain United Kingdom

Central Contacts

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Krystle Rapchak

Role: CONTACT

+1 (312) 847-1289

Eric Bauer

Role: CONTACT

Facility Contacts

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Madeline Pak

Role: primary

714-456-8413

Kristina Pfeifer, MA

Role: primary

415-353-8440

Divya Bhaskar

Role: primary

916-342-7496

Kimberly Klipner, RN

Role: primary

302-344-1528

Debbie Berry

Role: primary

407-545-7610

Alex Rivera

Role: primary

407-293-1122 ext. 121

Jazmine Roman

Role: primary

678-528-0961

Katie Harris

Role: primary

502-629-5820 ext. 50920

Kym Abraham, BS

Role: primary

402-559-2977

Millie Stone, BA

Role: primary

646-962-4664

Isabella Henderson

Role: primary

540-327-8456

Gloria Pinero

Role: primary

919-613-0767

Mikael Polanco

Role: primary

336-716-8940

Samantha Ballard, BSN, RN, CPN

Role: primary

513-803-3177

Honglian Huang, PhD

Role: primary

216-445-2366

Rashmi Risbud, Mbio

Role: primary

215-419-3212

Victoria Henderson, BS

Role: primary

210-416-4163

Holly Lawrence

Role: primary

214-456-9561

Laura Beeler, BS

Role: primary

801-585-2121

Laurie Guidry, RN

Role: primary

206-987-0058

Dora Xiong

Role: primary

604-875-2345 ext. 7132

Sarah Healy, MSc

Role: primary

613-737-7600 ext. 1605

Laura MacDougall, PhD, CCRP

Role: primary

416-813-7996

Sarah Iskandarani, MD

Role: primary

416-603-5800 ext. 5906

Tinatin Nadiradze, MD

Role: primary

+995558320502

Tamar Gachechiladze, MD

Role: primary

+995598357909

Sopiko Digmelashvili, MD

Role: primary

+995551516066

Csilla Nemeth Sandorne

Role: primary

+0036206701789

Merő Gabriella, MD

Role: primary

+36 70 451 3768

Katarzyna Karpińska

Role: primary

+48 58 349 23 31

Anna Byjoś

Role: primary

+48 50 107 68 35

Alicja Goszczańska-Ciuchta

Role: primary

+48 60 444 80 43

Nuria Ventosino

Role: primary

+346620708323 ext. 7351

Angela Fraga

Role: primary

+34633160818

Bethan Phillips

Role: primary

+44 29 2184 7816

Jenna Green

Role: primary

0151 228 4811

Emeka Agbasi

Role: primary

+44 20 7405 9200

Stuart Gormley

Role: primary

+44 114 305 3236

Other Identifiers

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2024-518628-57-00

Identifier Type: CTIS

Identifier Source: secondary_id

EPX-100-001

Identifier Type: -

Identifier Source: org_study_id

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