Authors :
Presenting Author: Aurelie Hanin, PharmD, PhD – Yale University School of Medicine
Anthony Jimenez, BS – Post-graduate, Neurology, Yale University School of Medicine; Hannah Asbell, RN, BSN – Children’s Hospital Colorado; Seyhmus Aydemir, MD – Neurology – Weill Cornell Medicine; Mayssa Basha, MD – Wayne State University School of Medicine; Ayush Batra, MD – Northwestern University; Charlotte Damien, MD – Neurology – Universite Libre de Bruxelles; Gregory Day, MD – Mayo Clinic Jacksonville; Zutshi Deepti, MD – Wayne State University School of Medicine; Onome Eka, M.B.B.S, MPH. – Icahn School of Medicine at Mount Sinai; Krista Eschbach, MD – Children’s Hospital Colorado; Safoora Fatima, Mrs – Neurology – University of Wisconsin; Madeline Fields, MD – Icahn School of Medicine at Mount Sinai; Brandon Foreman, MD MS FACNS FNCS – University of Cincinnati Medical Center; Hiba Haider, MD – Emory University; Stephen Hantus, MD – Cleveland Clinic; Sara Hocker, MD – Mayo Clinic; Elizabeth Gerard, MD – Northwestern University; Teneille Gofton, MD – University Hospital London Health Sciences Centers; Amy Jongeling, MD – New York University; Mariel Kalkach Aparicio, MD – Neurology – University of Wisconsin; Padmaja Kandula, MD – Neurology – Weill Cornell Medicine; Peter Kang, MD, MSCI – Washington University School of Medicine & Barnes-Jewish Hospital; Karnig Kazazian, BS – University Hospital London Health Sciences Centers; Marissa Kellogg, MD – Portland VA Healthcare System (POR VAHCS), Oregon Health and Science University (OHSU); Minjee Kim, MD – Northwestern University; Jong Woo Lee, MD, PhD – Brigham and Women's Hospital; 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, Mrs – Brigham and Women's Hospital; Cederic Pimentel, MD – Emory University; Vineet Punia, MD – Cleveland Clinic; Alexandra Ramirez, BS – University of Cincinnati Medical Center; Claude Steriade, MD – New York University; Aaron Struck, MD – Neurology – University of Wisconsin; Olga Taraschenko, MD, PhD – Department of Neurological Sciences – University of Nebraska Medical Center; Andrew Treister, MD – Oregon Health and Science University (OHSU); Mark Wainwright, MD, PhD – Seattle Children's Hospital; Ji Yeoun Yoo, MD – Icahn School of Medicine at Mount Sinai; Daniel Zhou, MD – Neurology – University of Nebraska Medical Center; Safar Zahar, MD – Massachusetts General Hospital; Nicolas Gaspard, MD, PhD – Professor of Neurology, Neurology, Universite Libre de Bruxelles; Lawrence Hirsch, MD – Professor of Neurology, Neurology, Yale University School of Medicine
Rationale:
New-Onset Refractory Status Epilepticus (NORSE) is a rare and often devastating condition occurring in children or adults without active epilepsy and without a clear acute or active structural, toxic, or metabolic cause identified in the first days. Guidance on appropriate treatment stems from case reports and case series, and a recent publication of consensus-based recommendations for the management of NORSE.
Here, we investigate if the management changed over time and differs based on age, clinical features, or etiology.
Methods:
Sixty five patients were enrolled by 24 centers in the NORSE/FIRES biorepository at Yale between December 2017 and March 2023. Patients were divided into three groups according to the year of SE onset: 2017-2019 (1st group, n=21), 2020-2021 (2nd group, n=22), and 2022-2023 (3rd group, n=22). Patients of the first and third groups were compared. Using all patients, we also compared pediatric versus adult ( >18 years old), cryptogenic NORSE versus identified etiology, and patients with NORSE who had a preceding febrile illness (defined as FIRES) versus non-FIRES.
Results:
Patients had a median age of 32 years (range 2-87) and included 11 pediatric cases (2-16 years old). Thirty-two patients (49%) qualified as FIRES. An etiology was found for 15 patients (23%).
Patients received a median of two continuous IV anti-seizure medications (ASM) and six conventional ASM. 22% of patients died, and patients had a median GOS-E of three (lower severe disability) at discharge, and five (lower moderate disability) during follow-up, without differences over the years or according to SE etiology. 83% of the patients received at least one immunotherapy, with the first line administered after a median of three days. 45% of the patients received second-line immunotherapy after a median of 14 days: rituximab 22%, anakinra 22%, and tocilizumab 15%.
Patients enrolled in 2022-2023 more often received anakinra (45% vs 5%, p=0.0068) and a ketogenic diet (50% vs 5%, p=0.0030) than patients from 2017-2019. The first-line and second-line immunotherapies were started within three days and two weeks for both groups.
Anakinra was more frequently prescribed for pediatric cases compared to adults (55% vs 15%, p=0.012), while no pediatric case received plasmapheresis (46% vs 0%, p=0.013). Patients with FIRES were more frequently treated with second-line immunotherapy, notably anakinra, than patients with non-FIRES (all immunotherapy: 66% vs 24%, p=0.0019; anakinra 41% vs 3%, p< 0.001). SE management did not change according to SE etiology (cryptogenic versus identified etiology).