A Double-blind Study Evaluating the Efficacy, Safety, and Tolerability of Zorevunersen in Patients With Dravet Syndrome

NCT ID: NCT06872125

Last Updated: 2026-02-09

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

170 participants

Study Classification

INTERVENTIONAL

Study Start Date

2025-06-04

Study Completion Date

2028-10-31

Brief Summary

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The purpose of the study is to evaluate the efficacy, safety, and tolerability of zorevunersen in Patients with Dravet syndrome.

Detailed Description

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Zorevunersen is an investigational new medicine for the treatment of Dravet syndrome. It is an antisense oligonucleotide (ASO) that is intended to increase the level of productive SCN1A messenger RNA (mRNA) and consequently increase the expression of the sodium channel Nav1.1 protein. This RNA-based approach is not gene therapy, but rather RNA modulation, as it does not manipulate nor insert genetic deoxyribonucleic acid (DNA).

Zorevunersen is designed to upregulate Nav1.1 protein expression from the nonmutant (wild-type) copy of the SCN1A gene to restore physiological Nav1.1 levels. Nav1.1 levels are reduced in people with Dravet syndrome.

This is a global, multicenter, randomized, double-blind, sham-controlled, parallel group Phase 3 study to assess the efficacy, safety, and tolerability of zorevunersen in patients with Dravet syndrome. The study duration and endpoints are designed to evaluate the potential of zorevunersen for disease modification. The study consists of two parts, Treatment Period 1 and Treatment Period 2. The primary and secondary endpoints will be assessed at the conclusion of Treatment Period 1. These endpoints will be assessed again at the end of Treatment Period 2. The primary endpoint is the change from baseline in major motor seizure frequency. Secondary endpoints include the change in behavior and cognition, clinical status, and health-related quality of life in patients with Dravet syndrome.

Patients will have the opportunity to enroll in an open label extension study and receive zorevunersen if they meet eligibility criteria at the end of the study.

Conditions

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Dravet Syndrome

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

QUADRUPLE

Participants Caregivers Investigators Outcome Assessors

Study Groups

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Zorevunersen

Eligible patients will be randomly assigned in a 1:1 ratio to zorevunersen:sham in Treatment Period 1 (approximately 52 weeks). Upon the completion of Treatment Period 1 all eligible patients, will enter Treatment Period 2 and receive zorevunersen, regardless of initial treatment assignment.

Group Type EXPERIMENTAL

zorevunersen

Intervention Type DRUG

Treatment Period 1: Zorevunersen group will receive study drug by intrathecal (IT) administration on Day 1 (after the 8-week Baseline Period), Day 57 (Week 8), Day 169 (Week 24), and Day 281 (Week 40) at a dose level of 70 mg on Day 1 and Day 57, and 45 mg on Day 169 and Day 281.

Treatment Period 2: Group assigned to zorevunersen in Treatment Period 1 will receive 45 mg of zorevunersen on Day 393 (Week 56), Day 477 (Week 68), and Day 589 (Week 84).

Sham Comparator

Eligible patients will be randomly assigned in a 1:1 ratio to zorevunersen:sham

Group Type SHAM_COMPARATOR

Sham Comparator

Intervention Type OTHER

Treatment Period 1: Sham group will not have drug administered. Sham group will have a procedure intended to mimic the drug administration.

Treatment Period 2: Group assigned to sham in Treatment Period 1 will receive 70 mg of zorevunersen on Day 393 (Week 56) and on Day 477 (Week 68), and 45 mg of zorevunersen Day 589 (Week 84).

Interventions

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zorevunersen

Treatment Period 1: Zorevunersen group will receive study drug by intrathecal (IT) administration on Day 1 (after the 8-week Baseline Period), Day 57 (Week 8), Day 169 (Week 24), and Day 281 (Week 40) at a dose level of 70 mg on Day 1 and Day 57, and 45 mg on Day 169 and Day 281.

Treatment Period 2: Group assigned to zorevunersen in Treatment Period 1 will receive 45 mg of zorevunersen on Day 393 (Week 56), Day 477 (Week 68), and Day 589 (Week 84).

Intervention Type DRUG

Sham Comparator

Treatment Period 1: Sham group will not have drug administered. Sham group will have a procedure intended to mimic the drug administration.

Treatment Period 2: Group assigned to sham in Treatment Period 1 will receive 70 mg of zorevunersen on Day 393 (Week 56) and on Day 477 (Week 68), and 45 mg of zorevunersen Day 589 (Week 84).

Intervention Type OTHER

Eligibility Criteria

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Inclusion Criteria

1. Patients must be ≥2 and \<18 years of age.
2. Patients must have a clinical diagnosis of DS confirmed by the Epilepsy Study Consortium, Inc. (ESCI) and as defined by:

Onset, prior to 12 months (inclusive, \<13 months), of age, of recurrent focal with motor signs, hemiclonic, or generalized tonic-clonic seizures. No other known etiology causing clinical DS manifestations..
3. Patient must have a documented pathogenic, likely pathogenic variant, or variant of uncertain significance in the sodium voltage-gated channel type 1 alpha subunit (SCN1A) gene. Patients who have SCN1A testing results of Negative (no variants identified) cannot be randomized.
4. Patient must experience the required number of major motor seizures during the 6-week Observation Period. Major motor seizure types included are Seizure types included in counts are Hemiclonic, Focal with Motor Signs, Focal to Bilateral Tonic-Clonic, Generalized Tonic-Clonic, Tonic, Tonic/Atonic (Drop Attacks with fall or risk of fall), and Bilateral Clonic.
5. Patient must have used at least 2 prior interventions for seizures. These can include anti-seizure medications (ASMs), ketogenic diet and/or vagus nerve stimulation (VNS) with either lack of adequate seizure control or discontinued due to an AE(s). These interventions can be ongoing therapies.
6. Patient must be taking at least one ASM. Benzodiazepines or ASMs used on a standing basis (i.e., not as needed \[PRN\]) for any indication will be considered an ASM.
7. Patients' maintenance ASMs and interventions for seizures (i.e., ketogenic diet or VNS), as well as any marijuana- or cannabinoid-based products, must have been stable (unless adjusted for weight) during the Baseline Period.

Exclusion Criteria

1. Patient has documented variant in the SCN1A gene associated with gain-of-function
2. Patient is currently treated with a maintenance ASM acting primarily as a sodium channel blocker, including but not limited to phenytoin, carbamazepine, oxcarbazepine, lamotrigine, lacosamide, rufinamide, or cenobamate, given the mechanism of action of zorevunersen.
3. Patient is currently treated with neuromodulation techniques (e.g., responsive neurostimulation, deep brain stimulation, or transcranial magnetic stimulation), with the exception of VNS.
4. Patient has emergence of a new seizure type or reemergence of a past seizure type (seizure types that last occurred more than 12 months before Screening Visit A) during the Baseline Period, or has more than 1 hospitalization for seizures during the Baseline Period.
Minimum Eligible Age

2 Years

Maximum Eligible Age

17 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Stoke Therapeutics, Inc

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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Phoenix Children's Hospital

Phoenix, Arizona, United States

Site Status RECRUITING

Arkansas Children's Hospital

Little Rock, Arkansas, United States

Site Status RECRUITING

Cedars Sinai Medical Center

Los Angeles, California, United States

Site Status RECRUITING

Children's Hospital of Orange County

Orange, California, United States

Site Status RECRUITING

USCF Medical Center

San Francisco, California, United States

Site Status RECRUITING

Children's Hospital Colorado

Aurora, Colorado, United States

Site Status RECRUITING

Children's National Medical Center

Washington D.C., District of Columbia, United States

Site Status RECRUITING

Nemours Children's Health

Jacksonville, Florida, United States

Site Status NOT_YET_RECRUITING

Nicklaus Children's Hospital

Miami, Florida, United States

Site Status RECRUITING

Advent Health Neuroscience Research Institute

Orlando, Florida, United States

Site Status RECRUITING

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

Chicago, Illinois, United States

Site Status RECRUITING

University of Iowa Hospital and Clinics

Iowa City, Iowa, United States

Site Status NOT_YET_RECRUITING

Massachusetts General Hospital

Boston, Massachusetts, United States

Site Status RECRUITING

Boston Children's Hospital

Boston, Massachusetts, United States

Site Status NOT_YET_RECRUITING

CS Mott Children's Hospital

Ann Arbor, Michigan, United States

Site Status RECRUITING

Mayo Clinic

Rochester, Minnesota, United States

Site Status RECRUITING

Washington University in St. Louis School of Medicine

St Louis, Missouri, United States

Site Status NOT_YET_RECRUITING

NYU Langone Health

New York, New York, United States

Site Status RECRUITING

Weill Cornell Medicine

New York, New York, United States

Site Status RECRUITING

University of Rochester Medical Center

Rochester, New York, United States

Site Status RECRUITING

University of North Carolina at Chapel Hill

Chapel Hill, North Carolina, United States

Site Status RECRUITING

Duke University Health System

Durham, North Carolina, United States

Site Status RECRUITING

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, United States

Site Status NOT_YET_RECRUITING

Cleveland Clinic

Cleveland, Ohio, United States

Site Status RECRUITING

Nationwide Children's Hospital

Columbus, Ohio, United States

Site Status RECRUITING

Oregon Health & Science University (OHSU)

Portland, Oregon, United States

Site Status RECRUITING

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Site Status NOT_YET_RECRUITING

LeBonheur Children's Hospital

Memphis, Tennessee, United States

Site Status RECRUITING

Cook Children's Medical Center

Fort Worth, Texas, United States

Site Status RECRUITING

Texas Children's Hospital

Houston, Texas, United States

Site Status RECRUITING

University of Utah Primary Children's Hospital

Salt Lake City, Utah, United States

Site Status RECRUITING

UVA Health

Charlottesville, Virginia, United States

Site Status RECRUITING

Fukuoka Children's Hospital

Fukuoka, , Japan

Site Status RECRUITING

Hokkaido University Hospital

Hokkaido, , Japan

Site Status RECRUITING

Kyoto University Hospital

Kyoto, , Japan

Site Status RECRUITING

Nagoya University Hospital

Nagoya, , Japan

Site Status RECRUITING

National Hospital Organization Nishi Niigata Central Hospital

Niigata, , Japan

Site Status RECRUITING

Okayama University Hospital

Okayama, , Japan

Site Status RECRUITING

Osaka City General Hospital

Osaka, , Japan

Site Status NOT_YET_RECRUITING

Jichi Medical University Hospital

Shimotsuke, , Japan

Site Status RECRUITING

NHO Shizuoka

Shizuoka, , Japan

Site Status RECRUITING

National Center of Neurology and Psychiatry

Tokyo, , Japan

Site Status RECRUITING

Yokohama City University Medical Center

Yokohama, , Japan

Site Status RECRUITING

Royal Hospital for Children

Glasgow, , United Kingdom

Site Status RECRUITING

Great Ormond Street Hospital for Children

London, , United Kingdom

Site Status NOT_YET_RECRUITING

Sheffield Children's Hospital

Sheffield, , United Kingdom

Site Status RECRUITING

Countries

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United States Japan United Kingdom

Central Contacts

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Emperor Information Center

Role: CONTACT

1-781-430-8200

Facility Contacts

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Nigel Negm

Role: primary

602-933-1169

Angus Wilfong, MD

Role: backup

Debopam Samanta, MD

Role: primary

501-364-3710

Andrea Sellew

Role: backup

Research Assistant

Role: primary

Maija-Riikka Steenari, MD

Role: primary

714-509-8972

Kaitlyn Sherer

Role: primary

415-353-2437

Antoinette Swanson

Role: backup

Ebonee Hayes

Role: primary

720-777-8008

Gabrielle Brockmeyer

Role: backup

720-777-2382

Chloe Hooker

Role: primary

202-476-6809

Emily Bush

Role: primary

904-697-3015

Dainelys Pena Rodriguez

Role: primary

786-624-3547

Ammar Hussain, MD

Role: primary

407-609-9040

Sofia Mirshed

Role: primary

312-227-4525

Enrique Rojas

Role: backup

312-227-2532

Michael Ciliberto, MD

Role: primary

319-353-8375

Giovi Hersch

Role: primary

617-726-2177

Christelle Moufawad El Achkar, MD

Role: primary

617-355-7970

Stephanie Rau

Role: primary

734-232-8474

Elaine Wirrell, MD

Role: primary

507-266-0774

Oleg Lobanov, MD

Role: primary

Jack Carter

Role: primary

646-558-0839

Natalie Wayland

Role: primary

646-962-3023

Cate Concannon

Role: primary

585-275-0404

Yulissa Gonzalez

Role: primary

919-843-3516

Gloria Pinero

Role: primary

919-613-0767

Kelly Kremer, MD

Role: primary

513-636-8980

Honglian Huang

Role: primary

216-445-2366

Anup Patel, MD

Role: primary

614-722-4625

Colin Roberts, MD

Role: primary

503-494-0879

Jennie Minnick

Role: primary

215-590-1719

James Wheless, MD

Role: primary

901-287-5325

Dianna Grado

Role: primary

682-885-2844

Scott Perry, MD

Role: backup

Anuranjita Nayak, MD

Role: primary

832-822-2778

Ryan Kennington

Role: primary

801-587-0833

Kimberlee Meegan

Role: primary

434-243-2040

Research Assistant

Role: primary

Research Assistant

Role: primary

Research Assistant

Role: primary

Research Assistant

Role: primary

Research Assistant

Role: primary

Research Assistant

Role: primary

Research Assistant

Role: primary

Research Assistant

Role: primary

Research Assistant

Role: primary

Andreas Brunklaus, MD

Role: primary

+0141-232-7600

Helen Cross, MD

Role: primary

+442-070-405-9200

Archana Desurkar, MD

Role: primary

+441-140-276-1111

Other Identifiers

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2024-519555-28

Identifier Type: EUDRACT_NUMBER

Identifier Source: secondary_id

STK-001-DS-301

Identifier Type: -

Identifier Source: org_study_id

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