Authors :
Presenting Author: Anthony Jimenez, BS – Yale University
Aurélie Hanin, PharmD, PhD – Neurology – Yale Univeristy; Margaret Gopaul, PhD, MSCP – Yale University; Hannah Asbell, RN, BSN – Children's Hospital Colorado; Seyhmus Aydemir, MD – Weill Cornell Medicine; Maysaa Basha, MD – Wayne State University; Ayush Batra, MD – Northwestern University; Charlotte Damien, MD – Universite Libre de Bruxelles; Gregory Day, MD – Washington University School of Medicine; Zutshi Deepti, MD – Wayne State University School of Medicine; Onome Eka, MBBS, MPH – Icahn School of Medicine at Mount Sinai; Krista Eschbach, MD – Children's Hospital Colorado; Safoora Fatima, MD – University of Wisconsin; Madeline Fields, MD – Icahn School of Medicine at Mount Sinai; Brandon Foreman, MD – University of Cincinnati Department of Neurology and Rehabilitation Medicine; Elizabeth Gerard, MD – Northwestern University; Teneille Gofton, MD – London Health Sciences Centers; Hiba Haider, MD – Emory University; Stephen Hantus, MD – Cleveland Clinic; Sara Hocker, MD – Mayo Clinic; Amy Jongeling, MD – New York University; Mariel Kalkach Aparicio, MD – University of Wisconsin; Padmaja Kandula, MD – Weill Cornell Medicine; Peter Kang, MD – Washington University School of Medicine; Karnig Kazazian, BSc – London Health Sciences Centers; Marissa Kellogg, MD – Oregon Health and Science University; Minjee Kim, MD – Northwestern University; Lara Marcuse, MD – Icahn School of Medicine at Mount Sinai; Christopher McGraw, MD – Massachusetts General Hospital; Wazim Mohamed, MD – Wayne State University School of Medicine; Janet Orozco, MD, PhD – Brigham and Women's Hospital; Cederic Pimentel, MD – Emory University; Vineet Punia, MD – Cleveland Clinic; Alexandra Ramirez, BS – University of Cincinnati Department of Neurology and Rehabilitation Medicine; Claude Steriade, MD – New York University; Aaron Struck, MD – University of Wisconsin; Olga Taraschenko, MD,PhD – University of Nebraska Medical Center; Andrew Treister, MD – Oregon Health and Science University; Ji Yeoun Yoo, MD – Icahn School of Medicine at Mount Sinai; Daniel Zhou, MD – University of Nebraska Medical Center; Nicolas Gaspard, MD, PhD – Universite Libre de Bruxelles; Lawrence Hirsch, MD – Yale University
Rationale:
New-onset refractory status epilepticus (NORSE) is a rare and severe neurological condition defined as refractory status epilepticus (SE) that occurs in individuals without a history of epilepsy, and without an acute toxic, metabolic, or structural cause. Within the spectrum of NORSE, febrile infection-related epilepsy syndrome (FIRES) represents a subset in which the onset of SE is preceded by a febrile infection. Here, we analyze a large cohort of patients to determine if there are differences between FIRES and non-FIRES cases regarding demographics, clinical features, neuroimaging, and outcomes.
Methods:
Fifty-nine patients above 14 years of age were included, by 24 centers, in the prospective NORSE/FIRES biorepository study between December 2017 and March 2023. We first compared the 25 patients with FIRES to the 34 patients with non-FIRES. Next, we compared patients aged between 14 and 34 years (n=26) to patients aged over 55 years (n=20). Finally, we ran a matched-age group analysis to compare 19 patients with FIRES to 19 patients with non-FIRES.
Results:
Patients with FIRES experienced prodromes more frequently than non-FIRES patients (92% vs 59%, p=0.011). No difference was found for non-inflammatory related prodromes (i.e., behavioral changes and hallucinations). Similarly, no difference was noted in SE clinical type (convulsive or non-convulsive), etiology, or MRI findings regarding abnormal findings, T2/FLAIR cortical hyperintensities, or temporal lobe hyperintensities. FIRES patients had higher CSF white blood cell counts than non-FIRES: median of 10 vs 3 (p=0.0075), without difference for protein levels. While FIRES patients received more anti-seizure medications and immunotherapy than non-FIRES patients (p< 0.001 and p=0.009, respectively), no difference was found in outcomes.