Authors :
Karnig Kazazian, PhD – Western University
Nicolas Gaspard, MD, PhD – Hôpital Universitaire de Bruxelles
Lawrence Hirsch, MD – Yale University School of Medicine
Marissa Kellogg, MD, MPH, FAES – Portland VA HCS & Oregon Health & Science Univ (OHSU)
Nora Wong, PhD – NORSE Institute
Raquel Farias-Moeller, MD – Medical College Wisconsin
Krista Eschbach, MD – Children Hospital Colorado
Presenting Author: Teneille Gofton, MD – Schulich School of Medicine and Dentistry, Western University, London Health Sciences Center, Ontario, Canada
Rationale:
Since 2019, the New-Onset Refractory Status Epilepticus (NORSE) Family Registry has supported an international effort to systematically collect clinical and epidemiological data on individuals affected by NORSE and FIRES (Febrile Infection-Related Epilepsy Syndrome). NORSE is a clinical presentation that occurs in previously healthy children and adults. FIRES is a subcategory of NORSE defined by a preceding febrile illness. Despite increasing recognition, both mortality and morbidity rates are often high and data on long-term outcomes and survivorship remain limited. Here, we provide an overview of the Registry and summarize progress to date.
Methods:
Survivors, surrogate or substitute decision makers, and clinicians submitted patient data to the REDCap-based registry through the NORSE Institute website: https://www.norseinstitute.org/norse-registry-2. The registry collects detailed information on past medical history, clinical presentation, disease course, survivorship, clinical sequelae including epilepsy, cognition, behavioral issues, and quality of life, among other variables. Participants are invited to complete follow-up surveys for up to two years after the initial seizure presentation. Enrolment is available in multiple languages and will remain open until December 2025.
Results:
To date, 128 participants have enrolled in the study (ages 2–78 years; median age: 12.5 years; 50 females, 78 males) from 25 countries across 6 continents. Of these, 80% had FIRES, and 76% are survivors of NORSE/FIRES. More than 6 months after onset, most survivors report a high monthly seizure burden and continued use of at least three anti-seizure medications. Children were significantly more likely to experience a high seizure burden ( >12 seizures/ month) than adults (p< 0.001). The median quality of life score among survivors was 4.9 out of 10. Patients reported significantly worse mood compared to their pre-NORSE/FIRES baseline (p < 0.01), while changes in anxiety were not statistically