Citations 3
Acute and Long‐Term EEG and seizure characteristics in new onset refractory status epilepticus (NORSE)
Gilani, Kia , Hanin, Aurélie , Gaspard, Nicolas , Batra, Ayush , Behrndt, Laken, Day, Gregory S. , Demeret, Sophie , Eschbach, Krista , Foreman, Brandon , Gerard, Elizabeth E. , Gopaul, Margaret T., Hantus, Stephen , Cobos‐Hernandez, Carla, Jimenez, Anthony D. , Jongeling, Amy, Kandula, Padmaja , Kang, Peter, Kellogg, Marissa, Kim, Minjee , Farias‐Moeller, Raquel , Morales, M., Pimentel, Cederic M., Ramírez, Alexandra , Taraschenko, Olga , Zhou, Daniel J., Gofton, Teneille E , Haider, Hiba A , Kandula, Padmaja , Farias-Moeller, Raquel , Navarro, Vincent , Steriade, Claude , Struck, A F , Taraschenko, Olga , Wainwright, Mark S , Hirsch, Lawrence J , Yoo, Ji Yeoun , Eschbach, Krista , Gaspard, Nicolas , Day, Gregory S , Batra, Ayush , Hanin, Aurelie , Foreman, Brandon , Gerard, Elizabeth E , Kim, Minjee , Gofton, Teneille , Gopaul, Margaret , Haider, Hiba A. , Jongeling, Amy , Kazazian, Karnig , Kellogg, Marissa , Navarro, Vincent , Steriade, Claude , Struck, Aaron F. , Wainwright, Mark S. , Zhou, Daniel J., Hirsch, Lawrence J. , Yoo, Ji Yeoun
Epilepsia | June 26, 2026 | Volume: No volume available | Issue: No issue available | Pages: No page available
Abstract Objective This study was undertaken to examine acute and chronic electroencephalographic (EEG) and seizure characteristics in new onset refractory status epilepticus (NORSE). Methods Multicenter inpatient and follow‐up clinical and EEG data were analyzed using International League Against Epilepsy definitions and American Clinical Neurophysiology Society (ACNS) EEG terminology. Results Forty‐four patients were included from the Yale NORSE/FIRES Biorepository (median age = 29 years, 33 female, 38 cryptogenic). Nine hundred twenty inpatient EEG days were reviewed (median = 13.5 days/patient). Presenting status epilepticus (SE) types included 24 convulsive SE (CSE), five focal–motor (FMSE), and 15 nonconvulsive (NCSE). Of 39 patients with post discharge follow‐up (median = 14.7 months), 61.5% ( n = 24) had seizures and required more antiseizure medications than seizure‐free patients (median = 4 vs. 1, p < .001). Inpatient EEG captured epileptiform discharges in all and periodic discharges in 38 patients. Seizures were captured in 38 patients: 33 electrographic (11 only electrographic) and 27 electroclinical. Total seizure burden ranged 6.5–29 615.0 min (median = 50.7). Seizures fulfilling ACNS EEG criteria for SE were captured in 20 patients; two had CSE and the remainder NCSE with coma, nine of whom also had FMSE. Inpatient seizure days (median = 50.0 vs. 15.5%, p = .004) and seizure burden (median = 42.9 vs. 0 min, p = .009) were higher in the first half of monitored days. Interictal findings were equally present and did not predict postdischarge seizures, whereas electroclinical seizures (90.9% vs. 45.5%, p = .037), seizure burden (median = 116.8 vs. 32.5 min, p = .045), and proportion of seizure days (36.3% vs. 20.0%, p = .037) did. Among 12 patients with follow‐up EEGs (median = 19.6 months post onset), posterior‐dominant rhythm (PDR) returned in nine; five had periodic discharges, and three had seizure captured. Significance Seizures are most commonly convulsive upon presentation and nonconvulsive or clinically subtle throughout the inpatient course in NORSE. Inpatient seizures, but not interictal abnormalities, occur earlier in the inpatient course and presence of electroclinical seizures, seizure burden, and proportion of seizure days but not interictal findings are associated with postdischarge seizures. Although return of PDR reflects neurological recovery, seizure persistence and EEG abnormalities post‐NORSE are common.
Identification of Distinct Biological Groups of Patients With Cryptogenic NORSE via Inflammatory Profiling
Guillemaud, Martin , Chávez, Mario , Kobeissy, Firas , Vezzani, Annamaria , Jimenez, Anthony D. , Basha, Maysaa , Batra, Ayush , Demeret, Sophie , Eka, Onome , Gaspard, Nicolas , Haider, Hiba A. , Hantus, Stephen , Aparicio, Mariel Kalkach , Kandula, Padmaja , Kim, Minjee , Mellor, Andrew L. , Mohamed, Wazim, Morales, M., Pimentel, Cederic M., Taraschenko, Olga , Torcida, Nathan , Wainwright, Mark S. , Yoo, Ji Yeoun , Hanin, Aurélie , Basha, Maysaa M , Foreman, Brandon , Gaspard, Nicolas , Gerard, Elizabeth E , Jongeling, Amy , Kalkach Aparicio, Mariel , Kazazian, Karnig , Kim, Minjee , Lai, Yi-Chen , Marois, Clémence , Morales, Mikaela , Steriade, Claude , Taraschenko, Olga , Torcida Sedano, Nathan , Wainwright, Mark S , Navarro, Vincent , Hanin, Aurelie , Vezzani, Annamaria , Batra, Ayush , Haider, Hiba A , Yoo, Ji Yeoun , Kobeissy, Firas , Eschbach, Krista , Foreman, Brandon , Gerard, Elizabeth E. , Gofton, Teneille , Howe, Charles L. , Kazazian, Karnig , Lai, Yi‐Chen , Ramirez, Alexandra M., Steriade, Claude , Struck, Aaron F. , Wang, Kevin , Navarro, Vincent , Hirsch, Lawrence J.
Neurology Neuroimmunology & Neuroinflammation | May 7, 2025 | Volume: 12 | Issue: 4 | Pages: No page available
Trends in management of patients with new‐onset refractory status epilepticus (NORSE) from 2016 to 2023: An interim analysis
Hanin, Aurélie , Jimenez, Anthony D. , Asbell, Hannah, Aydemir, Seyhmus, Basha, Maysaa , Batra, Ayush , Damien, Charlotte , Day, Gregory S. , Eka, Onome , Eschbach, Krista , Fatima, Safoora , Fields, Madeline , Haider, Hiba A. , Hantus, Stephen , Hocker, Sara E. , Aparicio, Mariel Kalkach , Kandula, Padmaja , Kang, Peter , Kim, Minjee , Marcuse, Lara , Mohamed, Wazim, Orozco, Janet, Pimentel, Cederic M., Punia, Vineet , Taraschenko, Olga , Treister, Andrew , Wainwright, Mark S. , Yoo, Ji Yeoun , Zafar, Sahar F. , Zhou, Daniel J. , Zutshi, Deepti , Gaspard, Nicolas , Basha, Maysaa M , Batra, Ayush , Eschbach, Krista , Gofton, Teneille E , Haider, Hiba A , Hocker, Sara, Kalkach Aparicio, Mariel , Kandula, Padmaja , Lee, Jong Woo , Mohamed, Wazim , Punia, Vineet , Taraschenko, Olga , Yoo, Ji Yeoun , Zafar, Sahar , Zutshi, Deepti , Hirsch, Lawrence J , Wainwright, Mark S , Gaspard, Nicolas , Day, Gregory S , Hanin, Aurelie , Kim, Minjee , Gopaul, Margaret , Foreman, Brandon , Gerard, Elizabeth E. , Gofton, Teneille , Jongeling, Amy , Kazazian, Karnig , Kellogg, Marissa , McGraw, Christopher Michael , Ramirez, Alexandra M., Steriade, Claude , Struck, Aaron F. , Hirsch, Lawrence J.
Epilepsia | June 5, 2024 | Volume: 65 | Issue: 8 | Pages: No page available
AbstractIn response to the evolving treatment landscape for new‐onset refractory status epilepticus (NORSE) and the publication of consensus recommendations in 2022, we conducted a comparative analysis of NORSE management over time. Seventy‐seven patients were enrolled by 32 centers, from July 2016 to August 2023, in the NORSE/FIRES biorepository at Yale. Immunotherapy was administered to 88% of patients after a median of 3 days, with 52% receiving second‐line immunotherapy after a median of 12 days (anakinra 29%, rituximab 25%, and tocilizumab 19%). There was an increase in the use of second‐line immunotherapies (odds ratio [OR] = 1.4, 95% CI = 1.1–1.8) and ketogenic diet (OR = 1.8, 95% CI = 1.3–2.6) over time. Specifically, patients from 2022 to 2023 more frequently received second‐line immunotherapy (69% vs 40%; OR = 3.3; 95% CI = 1.3–8.9)—particularly anakinra (50% vs 13%; OR = 6.5; 95% CI = 2.3–21.0), and the ketogenic diet (OR = 6.8; 95% CI = 2.5–20.1)—than those before 2022. Among the 27 patients who received anakinra and/or tocilizumab, earlier administration after status epilepticus onset correlated with a shorter duration of status epilepticus (ρ = .519, p = .005). Our findings indicate an evolution in NORSE management, emphasizing the increasing use of second‐line immunotherapies and the ketogenic diet. Future research will clarify the impact of these treatments and their timing on patient outcomes.