The NORSE/FIRES Family Registry: Comparing new-onset refractory status epilepticus (NORSE) without preceding fever to febrile infection-related epilepsy syndrome (FIRES) (P9-1.003)

Abstract

<h3>Objective:</h3> 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). <h3>Background:</h3> 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. <h3>Design/Methods:</h3> 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. <h3>Results:</h3> 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 &lt; 0.001; 19/28 or 68% adults, 40/41 or 98% children). Adults with FIRES more often had fatigue (p &lt; 0.01) compared to adults with NORSE without fever. Adults with FIRES more often had flu-like illness (p &lt; 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. <h3>Conclusions:</h3> 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. <b>Disclosure:</b> Mr. Kazazian has nothing to disclose. Prof. Gaspard has nothing to disclose. Dr. Hirsch has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Aquestive. Dr. Hirsch has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ceribell. Dr. Hirsch has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for marinus. Dr. Hirsch has received personal compensation in the range of $0-$499 for serving as a Consultant for Medtronic. Dr. Hirsch has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. Hirsch has received personal compensation in the range of $0-$499 for serving as a Consultant for Accure. Dr. Hirsch has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Neuropace. Dr. Hirsch has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Neurelis. Dr. Hirsch has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eisai. Dr. Hirsch has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Rafa Laboratories, Ltd. Dr. Hirsch has received publishing royalties from a publication relating to health care. Dr. Hirsch has received publishing royalties from a publication relating to health care. Dr. Hirsch has received personal compensation in the range of $500-$4,999 for serving as a Speaker with Neuropace. Dr. Hirsch has received personal compensation in the range of $500-$4,999 for serving as a Speaker with Natus. Dr. Hirsch has received personal compensation in the range of $500-$4,999 for serving as a speaker with UCB. Dr. Kellogg has nothing to disclose. The institution of Dr. Hocker has received research support from Yale University. Dr. Hocker has received publishing royalties from a publication relating to health care. Ms. Wong has nothing to disclose. Dr. Farias-Moeller has nothing to disclose. The institution of Dr. Eschbach has received research support from Neurocrine Biosciences. The institution of Dr. Eschbach has received research support from UCB Biopharma. Dr. Eschbach has a non-compensated relationship as a Medical Science Advisory Board Member with NORSE Institute that is relevant to AAN interests or activities. The institution of Dr. Gofton has received research support from Government of Canada. The institution of Dr. Gofton has received research support from Canadian Institutes of Health Research. The institution of Dr. Gofton has received research support from Academic Medical Organisation of Southwestern Ontario. Dr. Gofton has a non-compensated relationship as a Medical Advisory Board Member with NORSE Institute that is relevant to AAN interests or activities.