RecruitingPhase 3NCT06872125

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

EMPEROR: A Multicenter, Randomized, Double-blind, Sham-controlled, Parallel Group, Phase 3 Study Evaluating the Efficacy, Safety, and Tolerability of Zorevunersen (STK-001) in Patients With Dravet Syndrome


Sponsor

Stoke Therapeutics, Inc

Enrollment

170 participants

Start Date

Jun 4, 2025

Study Type

INTERVENTIONAL

Conditions

Summary

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


Eligibility

Min Age: 2 YearsMax Age: 17 Years

Inclusion Criteria8

  • Patients must be ≥2 and \<18 years of age.
  • 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..
  • 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.
  • 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.
  • 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.
  • 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.
  • 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 Criteria4

  • Patient has documented variant in the SCN1A gene associated with gain-of-function
  • 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.
  • Patient is currently treated with neuromodulation techniques (e.g., responsive neurostimulation, deep brain stimulation, or transcranial magnetic stimulation), with the exception of VNS.
  • 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.

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Interventions

DRUGzorevunersen

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).

OTHERSham 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).


Locations(45)

Phoenix Children's Hospital

Phoenix, Arizona, United States

Arkansas Children's Hospital

Little Rock, Arkansas, United States

Cedars Sinai Medical Center

Los Angeles, California, United States

Children's Hospital of Orange County

Orange, California, United States

USCF Medical Center

San Francisco, California, United States

Children's Hospital Colorado

Aurora, Colorado, United States

Children's National Medical Center

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

Nemours Children's Health

Jacksonville, Florida, United States

Nicklaus Children's Hospital

Miami, Florida, United States

Advent Health Neuroscience Research Institute

Orlando, Florida, United States

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

Chicago, Illinois, United States

University of Iowa Hospital and Clinics

Iowa City, Iowa, United States

Massachusetts General Hospital

Boston, Massachusetts, United States

Boston Children's Hospital

Boston, Massachusetts, United States

CS Mott Children's Hospital

Ann Arbor, Michigan, United States

Mayo Clinic

Rochester, Minnesota, United States

Washington University in St. Louis School of Medicine

St Louis, Missouri, United States

NYU Langone Health

New York, New York, United States

Weill Cornell Medicine

New York, New York, United States

University of Rochester Medical Center

Rochester, New York, United States

University of North Carolina at Chapel Hill

Chapel Hill, North Carolina, United States

Duke University Health System

Durham, North Carolina, United States

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, United States

Cleveland Clinic

Cleveland, Ohio, United States

Nationwide Children's Hospital

Columbus, Ohio, United States

Oregon Health & Science University (OHSU)

Portland, Oregon, United States

LeBonheur Children's Hospital

Memphis, Tennessee, United States

Cook Children's Medical Center

Fort Worth, Texas, United States

Texas Children's Hospital

Houston, Texas, United States

University of Utah Primary Children's Hospital

Salt Lake City, Utah, United States

UVA Health

Charlottesville, Virginia, United States

Fukuoka Children's Hospital

Fukuoka, Japan

Hokkaido University Hospital

Hokkaido, Japan

Kyoto University Hospital

Kyoto, Japan

Nagoya University Hospital

Nagoya, Japan

National Hospital Organization Nishi Niigata Central Hospital

Niigata, Japan

Okayama University Hospital

Okayama, Japan

Osaka City General Hospital

Osaka, Japan

Jichi Medical University Hospital

Shimotsuke, Japan

NHO Shizuoka

Shizuoka, Japan

National Center of Neurology and Psychiatry

Tokyo, Japan

Yokohama City University Medical Center

Yokohama, Japan

Royal Hospital for Children

Glasgow, United Kingdom

Great Ormond Street Hospital for Children

London, United Kingdom

Sheffield Children's Hospital

Sheffield, United Kingdom

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