Initial Findings from the New-Onset Refractory Status Epilepticus (NORSE/FIRES) Family Registry
Abstract number :
3.468
Submission category :
4. Clinical Epilepsy / 4D. Prognosis
Year :
2023
Submission ID :
1453
Source :
www.aesnet.org
Presentation date :
12/4/2023 12:00:00 AM
Published date :
Authors :
Presenting Author: Teneille Gofton, MD FRCPC – Western University
Karnig Kazazian, BSc – Western University; Nicolas Gaspard, MD PhD – Université Libre de Bruxelles–Hôpital Erasme; Lawrence Hirsch, MD – Yale Comprehensive Epilepsy Center; Marissa Kellogg, MD – Oregon Health and Science University; Sara Hocker, MD – Saint Luke's Health System; Nora Wong, PhD – NORSE Institute; Raquel Farias-Moeller, MD – Medical College of Wisconsin; Krista Eschbach, MD – University of Colorado, Children's Hospital Colorado
Rationale:
New-Onset Refractory Status Epilepticus (NORSE) Family Registry contributes to an international and systematic effort to collect a range of clinical and epidemiological information on individuals affected by NORSE and FIRES (Febrile Infection-related Epilepsy Syndrome) worldwide. NORSE is a clinical presentation affecting previously healthy children and adults. FIRES is a subcategory of NORSE and applies when a preceding fever occurred. Information pertaining to disease course and survivorship remains limited and mortality and morbidity are variable but often high.
Methods:
Survivors, surrogate/substitute decision makers, and clinicians can enter patient data into the REDCap-based registry via a link on the NORSE Institute website: https://www.norseinstitute.org/norse-registry-2 . Information collected in this study includes past medical history, clinical presentation, disease course, survivorship, clinical sequelae and quality of life, among other variables. Participants are invited to complete follow-up surveys for up to two years following clinical presentation of seizures. Enrollment is ongoing in multiple languages and will remain open until 2025.
Results:
To date, 103 participants have enrolled in this study (2-78 years of age, mean: 17.1y; 41 females and 62 males) from 24 different countries across six continents. A total of 77% of participants are survivors of NORSE/FIRES. At < six months after the onset of NORSE/FIRES, survivors experience a high monthly seizure burden and remain on >3 anti-seizure medications. A total of 69% of children surviving FIRES, have >12 seizures/month (20/29 children with FIRES) compared to 14% of adults surviving FIRES (2/14 adults with FIRES; p< 0.001). The median quality of life amongst survivors was 4.4/10 in children and 5.0/10 for adults.
Clinical Epilepsy