The NORSE/FIRES Family Registry: Comparing new-onset refractory status epilepticus (NORSE) without preceding fever to febrile infection-related epilepsy syndrome (FIRES)
Karnig Kazazian1, Nicolas Gaspard2, Lawrence Hirsch3, Marissa Kellogg4, Sara Hocker5, Nora Wong6, Raquel Farias-Moeller7, Krista Eschbach8, Teneille Gofton9
1Western University, 2Université Libre de Bruxelles–Hôpital Erasme, 3Yale University Comprehensive Epilepsy Center, 4VA Portland Healthcare System, Dept of Neurology, 5Mayo Clinic, 6NORSE Institute, 7Children's National Medical Center, 8Childrens Hospital Colorado, 9London Health Sciences Centre
Objective:
In this work, we compare differences in clinical presentation, survivorship, and quality of life in people with new-onset refractory status epilepticus (NORSE) without preceding fever to febrile infection-related epilepsy syndrome (FIRES).
Background:
NORSE is a clinical presentation affecting previously healthy adults and children. FIRES is a subcategory of NORSE and applies when a preceding fever occurs within 24h to 2 weeks prior to the onset of NORSE. FIRES is thought to be more common in children and robust data describing FIRES in adults is lacking.
Design/Methods:
Survivors, surrogates, and physicians can enter data into a registry via a link on the NORSE Institute Webpage (https://www.norseinstitute.org/norse-registry-2). The registry collects the following information: past medical history, clinical presentation, disease course, survivorship, clinical sequelae and quality of life, among others. Participants are invited to complete follow-up surveys for up to two years following clinical presentation of seizures.
Results:
To date, there are 69 participants and 46/69 are survivors in the registry. There are 28 adults (18 survivors) and 41 children (28 survivors). 60/69 (87%) patients had a preceding fever, meeting the criteria for FIRES, with significantly more children having a preceding fever (p < 0.001; 19/28 or 68% adults, 40/41 or 98% children). Adults with FIRES more often had fatigue (p < 0.01) compared to adults with NORSE without fever. Adults with FIRES more often had flu-like illness (p < 0.0001) as presenting symptoms compared to children with FIRES. The presence or absence of fever was not associated with the likelihood of survival, number of seizures per month or quality of life in adults.
Conclusions:
These data suggest that FIRES is common in adults and children presenting with NORSE. Further prospective epidemiologic studies will be important to determine the incidence and prevalence of adult FIRES and whether fever is relevant to management or outcomes associated with NORSE.