NCT07281027

Brief Summary

The goal of this clinical trial is to find out whether two existing medications-anakinra and tocilizumab-can effectively treat a rare and life-threatening brain condition called NORSE (New-Onset Refractory Status Epilepticus). NORSE causes continuous seizures in previously healthy children and adults and does not respond to standard treatments. It often leads to long-term disability or death. Doctors currently use anakinra and tocilizumab as second-line treatments when first-line therapies fail, but there is no clear evidence showing which drug works better or when it should be given. This study aims to answer those questions. The study will enroll patients across 33 hospitals in the United States, Canada, Europe, and Asia. It includes two groups:

  • Receive one of the two medications (depending on their group assignment).
  • Take part in follow-up assessments over the course of one year, including medical evaluations and surveys. Some participants may be followed annually beyond one year.
  • Optionally participate in a 60-minute interview to share their or their caregiver's experience with NORSE.

Trial Health

70
Monitor

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
438

participants targeted

Target at P50-P75 for phase_3

Timeline
54mo left

Started Mar 2026

Longer than P75 for phase_3

Geographic Reach
7 countries

33 active sites

Status
not yet recruiting

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

Study Progress4%
Mar 2026Sep 2030

First Submitted

Initial submission to the registry

November 21, 2025

Completed
24 days until next milestone

First Posted

Study publicly available on registry

December 15, 2025

Completed
3 months until next milestone

Study Start

First participant enrolled

March 1, 2026

Completed
4.1 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

March 31, 2030

Expected
6 months until next milestone

Study Completion

Last participant's last visit for all outcomes

September 30, 2030

Last Updated

December 15, 2025

Status Verified

December 1, 2025

Enrollment Period

4.1 years

First QC Date

November 21, 2025

Last Update Submit

December 3, 2025

Conditions

Keywords

refractory status epilepticusNORSEFIRESanakinratocilizumab

Outcome Measures

Primary Outcomes (1)

  • Glasgow Outcome Scale - Extended (GOS-E)

    The Glasgow Outcome Scale - Extended (GOS-E) is an 8-point scale used to measure global functional outcome. Participants are scored into one of the 8 categories: 1. Death, 2. Vegetative State, 3. Lower Severe Disability, 4. Upper Severe Disability, 5. Lower Moderate Disability, 6. Upper Moderate Disability, 7. Lower Good Recovery, 8. Upper Good Recovery.

    12 months

Secondary Outcomes (6)

  • Time to resolution of status epilepticus (SE)

    24 hours off anesthetic drips

  • Hospital length of stay

    12 months

  • Mortality

    up to 12 months

  • Number of serious adverse events attributed to anakinra or tocilizumab

    From hospitalization to 1 month after stopping treatment, up to 12 months

  • Number of participants Post-NORSE epilepsy

    12 months

  • +1 more secondary outcomes

Study Arms (2)

Randomized Controlled Trial (RCT) Cohort

ACTIVE COMPARATOR

A randomized controlled cohort (RCT) of anakinra vs. tocilizumab (targeted immunotherapies) started up to and including 7 days after the onset of status epilepticus (SE)

Drug: AnakinraDrug: Tocilizumab

Observational Cohort

OTHER

An observational cohort enrolling patients with acute cryptogenic NORSE who cannot be randomized or who are identified too late to be randomized by the end of day 7 .

Other: Standard medical treatment

Interventions

SOC will be followed , Suggested Dose: 10 mg/kg/day IV, divided into 4 daily doses (q6h) Maximum dose: 400 mg/day

Randomized Controlled Trial (RCT) Cohort

SOC will be followed, Suggested Dose: If \<30 kg: 12 mg/kg IV once every 2 weeks If ≥30 kg: 8 mg/kg IV once every 2 weeks Maximum dose: 800 mg per dose

Randomized Controlled Trial (RCT) Cohort

For patients who could not be randomized by day 7, standard clinical care will be followed and patients will be followed prospectively and observationally.

Observational Cohort

Eligibility Criteria

Age2 Years+
Sexall
Healthy VolunteersNo
Age GroupsChild (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

  • Age 2 and older.
  • In their usual state of health prior to their onset of SE.
  • Presenting with NORSE as defined in the consensus criteria:
  • Refractory SE (failed 2 appropriately used anti-seizure medications) in a patient without active epilepsy or other pre-existing relevant neurological disorder and without an acute or active structural, toxic, or metabolic cause found in the first 72 hours.
  • Includes patients with any RSE, not just super-refractory SE.
  • Includes patients who ultimately are discovered to have a known etiology (infectious, autoimmune, genetic, etc.), as well as those who remain cryptogenic.
  • Anakinra and/or tocilizumab are being planned or considered as part of standard clinical care.
  • The onset of SE was in the prior 7 days at the time of enrollment.

You may not qualify if:

  • Any acute or active systemic medical illness such as metastatic cancer, renal failure, hepatic failure, poorly controlled diabetes, etc., in the opinion of the investigators. If this is unclear, the study PI Dr. Hirsch will determine if this criterion is met.
  • Contraindication to either anakinra or tocilizumab as listed in the prescribing information:
  • Known hypersensitivity to E. Coli-derived proteins, anakinra, tocilizumab, or any component of the products
  • Active serious infection at the time of initiation
  • Concomitant use of TNF blocking agents; absolute neutrophil count \< 2000; platelet count \< 100,000 per mm³; or ALT or AST \> 1.5 X the upper limit of normal
  • Elevated risk of GI perforation.

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (33)

Barrow Institute

Phoenix, Arizona, 85013, United States

Location

Children's Hospital Colorado

Aurora, Colorado, 80045, United States

Location

Yale New Haven Hospital

New Haven, Connecticut, 06520, United States

Location

Children's National (DC)

Washington D.C., District of Columbia, 20010, United States

Location

University of Florida

Gainesville, Florida, 32611, United States

Location

University of Chicago

Chicago, Illinois, 60637, United States

Location

Northwestern University

Evanston, Illinois, 60208, United States

Location

Mass General (MGH)

Boston, Massachusetts, 02114, United States

Location

Beth Israel Deaconess

Boston, Massachusetts, 02215, United States

Location

Boston Children's Hospital

Boston, Massachusetts, 02215, United States

Location

Mayo Clinic

Rochester, Minnesota, 55905, United States

Location

University of Nebraska

Lincoln, Nebraska, 68588, United States

Location

New York University

New York, New York, 10012, United States

Location

Columbia University

New York, New York, 10027, United States

Location

Mount Sinai (NY)

New York, New York, 10029, United States

Location

University of Cincinnati

Cincinnati, Ohio, 45221, United States

Location

Cleveland Clinic

Cleveland, Ohio, 44195, United States

Location

Oregon Health and Science University

Portland, Oregon, 97239, United States

Location

Children's Hospital Philadelphia (CHOP)

Philadelphia, Pennsylvania, 19104, United States

Location

University of Pennsylvania

Philadelphia, Pennsylvania, 19104, United States

Location

UT Southwestern Medical Center

Dallas, Texas, 75390, United States

Location

Baylor/Texas Children's

Houston, Texas, 77030, United States

Location

Seattle Children's Hospital

Seattle, Washington, 98105, United States

Location

University of Wisconsin

Madison, Wisconsin, 53706, United States

Location

Medical College of Wisconsin

Milwaukee, Wisconsin, 53226, United States

Location

Western University

London, Canada

Location

Hospital for Sick Children

Toronto, Canada

Location

Salpêtrière

Paris, France

Location

University of Modena

Modena, Italy

Location

Seoul National University Hospital

Seoul, South Korea

Location

Karolinska Institute

Stockholm, Sweden

Location

Great Ormond Street Hospital

London, United Kingdom

Location

King's College

London, United Kingdom

Location

MeSH Terms

Conditions

Status Epilepticus

Interventions

Interleukin 1 Receptor Antagonist Proteintocilizumab

Condition Hierarchy (Ancestors)

SeizuresNeurologic ManifestationsNervous System DiseasesSigns and SymptomsPathological Conditions, Signs and Symptoms

Intervention Hierarchy (Ancestors)

CytokinesIntercellular Signaling Peptides and ProteinsPeptidesAmino Acids, Peptides, and ProteinsProteinsBiological Factors

Study Officials

  • Lawrence Hirsch, MD

    Yale University

    PRINCIPAL INVESTIGATOR

Central Study Contacts

Camalene Chrysostoum

CONTACT

Study Design

Study Type
interventional
Phase
phase 3
Allocation
RANDOMIZED
Masking
SINGLE
Who Masked
OUTCOMES ASSESSOR
Purpose
TREATMENT
Intervention Model
PARALLEL
Sponsor Type
OTHER
Responsible Party
PRINCIPAL INVESTIGATOR
PI Title
Professor of Neurology

Study Record Dates

First Submitted

November 21, 2025

First Posted

December 15, 2025

Study Start

March 1, 2026

Primary Completion (Estimated)

March 31, 2030

Study Completion (Estimated)

September 30, 2030

Last Updated

December 15, 2025

Record last verified: 2025-12

Data Sharing

IPD Sharing
Will share

Making the Full Data Package available to enable other researchers (including PCORI) to use it to re-analyze the data or for independent (i.e., novel) research analyses.

Locations