The NORSE/FIRES Family Registry: A comparison of pediatric and adult presentations and outcomes
Abstract number :
2.429
Submission category :
4. Clinical Epilepsy / 4A. Classification and Syndromes
Year :
2022
Submission ID :
2232909
Source :
www.aesnet.org
Presentation date :
12/4/2022 12:00:00 PM
Published date :
Nov 22, 2022, 05:28 AM
Authors :
Teneille Gofton, MD FRCPC CSCN(EEG) – 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 – Mayo Clinic; Nora Wong, PhD – NORSE Institute; Raquel Farias-Moeller, MD – Medical College of Wisconsin; Krista Eschbach, MD – Children's Hospital Colorado
This is a Late Breaking abstract
Rationale: New-onset refractory status epilepticus (NORSE) is a clinical presentation affecting previously healthy adults and children. Febrile infection-related epilepsy syndrome (FIRES) is a subcategory of NORSE and applies when a preceding fever occurs within 24 h 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.
Methods: Survivors, surrogate/substitute decision makers and physicians can enter patient data into the REDCap-based registry via a link on the NORSE Institute website (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. Enrollment is ongoing in multiple languages and will remain open until 2025. Statistical analysis was performed using Chi-squared and ANOVA tests.
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 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 prodromal fatigue (p < 0.01) compared to adults with NORSE without fever. No other significant differences were seen between adults with NORSE without fever versus adults with FIRES. Adults with FIRES more often had flu-like illness (p < 0.0001) as a presenting symptom compared to children with FIRES. When considering outcomes, the presence or absence of fever was not associated with the ICU length of stay, likelihood of survival, number of seizures per month or quality of life in adults. The effect of fever on outcomes in children could not be tested because only one child had NORSE without fever. Because of the self-reported study design, it is difficult to determine associations with etiology of NORSE, severity of status epilepticus or treatment administered in the acute phase._x000D_
Clinical Epilepsy