Gaspard N, Foreman BP, Alvarez V, Cabrera KC, Probasco JC, Jongeling AC, Meyers E, Espinera A, Haas KF, Schmitt SE, Gerard EE, Gofton T, Kaplan PW, Lee JW, Legros B, Szaflarski JP, Westover BM, LaRoche SM, Hirsch LJ, Care CEEGMRC (2015) New-onset refractory status epilepticus: Etiology, clinical features, and outcome. Neurology 85(18):1604–1613. https://doi.org/10.1212/WNL.0000000000001940

Article  PubMed  PubMed Central  Google Scholar 

Hirsch LJ, Gaspard N, van Baalen A, Nabbout R, Demeret S, Loddenkemper T, Navarro V, Specchio N, Lagae L, Rossetti AO, Hocker S, Gofton TE, Abend NS, Gilmore EJ, Hahn C, Khosravani H, Rosenow F, Trinka E (2018) Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. Epilepsia 59(4):739–744. https://doi.org/10.1111/epi.14016

Article  PubMed  Google Scholar 

Matthews E, Alkhachroum A, Massad N, Letchinger R, Doyle K, Claassen J, Thakur KT (2020) New-onset super-refractory status epilepticus: A case series of 26 patients. Neurology 95(16):e2280–e2285. https://doi.org/10.1212/WNL.0000000000010787

Article  PubMed  PubMed Central  Google Scholar 

Taraschenko O, Pavuluri S, Schmidt CM, Pulluru YR, Gupta N (2023) Seizure burden and neuropsychological outcomes of new-onset refractory status epilepticus: systematic review. Front Neurol 14:1095061. https://doi.org/10.3389/fneur.2023.1095061

Article  PubMed  PubMed Central  Google Scholar 

Kramer U, Chi CS, Lin KL, Specchio N, Sahin M, Olson H, Kluger G, van Baalen A (2011) Febrile infection-related epilepsy syndrome (FIRES): does duration of anesthesia affect outcome? Epilepsia 52(Suppl 8):28–30. https://doi.org/10.1111/j.1528-1167.2011.03230.x

Article  PubMed  Google Scholar 

Wu J, Lan X, Yan L, Hu Y, Hong S, Jiang L, Chen J (2021) A retrospective study of 92 children with new-onset refractory status epilepticus. Epilepsy Behav 125:108413. https://doi.org/10.1016/j.yebeh.2021.108413

Article  PubMed  Google Scholar 

Gaspard N, Hirsch LJ, Sculier C, Loddenkemper T, van Baalen A, Lancrenon J, Emmery M, Specchio N, Farias-Moeller R, Wong N, Nabbout R (2018) New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): State of the art and perspectives. Epilepsia 59(4):745–752. https://doi.org/10.1111/epi.14022

Article  PubMed  Google Scholar 

Gaspard N, Shorvon S (2023) Is NORSE a meaningful clinical entity? Epilepsy Behav 141:109097. https://doi.org/10.1016/j.yebeh.2023.109097

Article  PubMed  Google Scholar 

Lattanzi S, Leitinger M, Rocchi C, Salvemini S, Matricardi S, Brigo F, Meletti S, Trinka E (2022) Unraveling the enigma of new-onset refractory status epilepticus: a systematic review of aetiologies. Eur J Neurol 29(2):626–647. https://doi.org/10.1111/ene.15149

Article  PubMed  Google Scholar 

Tharmaraja T, Ho JSY, Neligan A, Rajakulendran S (2023) The etiology and mortality of new-onset refractory status epilepticus (NORSE) in adults: A systematic review and meta-analysis. Epilepsia 64(5):1113–1124. https://doi.org/10.1111/epi.17523

Article  PubMed  Google Scholar 

Clarkson BDS, LaFrance-Corey RG, Kahoud RJ, Farias-Moeller R, Payne ET, Howe CL (2019) Functional deficiency in endogenous interleukin‑1 receptor antagonist in patients with febrile infection-related epilepsy syndrome. Ann Neurol 85(4):526–537. https://doi.org/10.1002/ana.25439

Article  CAS  PubMed  PubMed Central  Google Scholar 

Saitoh M, Kobayashi K, Ohmori I, Tanaka Y, Tanaka K, Inoue T, Horino A, Ohmura K, Kumakura A, Takei Y, Hirabayashi S, Kajimoto M, Uchida T, Yamazaki S, Shiihara T, Kumagai T, Kasai M, Terashima H, Kubota M, Mizuguchi M (2016) Cytokine-related and sodium channel polymorphism as candidate predisposing factors for childhood encephalopathy FIRES/AERRPS. J Neurol Sci 368:272–276. https://doi.org/10.1016/j.jns.2016.07.040

Article  CAS  PubMed  Google Scholar 

Kothur K, Bandodkar S, Wienholt L, Chu S, Pope A, Gill D, Dale RC (2019) Etiology is the key determinant of neuroinflammation in epilepsy: Elevation of cerebrospinal fluid cytokines and chemokines in febrile infection-related epilepsy syndrome and febrile status epilepticus. Epilepsia 60(8):1678–1688. https://doi.org/10.1111/epi.16275

Article  CAS  PubMed  Google Scholar 

Sakuma H, Tanuma N, Kuki I, Takahashi Y, Shiomi M, Hayashi M (2015) Intrathecal overproduction of proinflammatory cytokines and chemokines in febrile infection-related refractory status epilepticus. J Neurol Neurosurg Psychiatry 86(7):820–822. https://doi.org/10.1136/jnnp-2014-309388

Article  PubMed  Google Scholar 

Wang D, Wu Y, Pan Y, Wang S, Liu G, Gao Y, Xu K (2023) Multi-proteomic analysis revealed distinct protein profiles in cerebrospinal fluid of patients between anti-NMDAR encephalitis NORSE and Cryptogenic NORSE. Mol Neurobiol 60(1):98–115. https://doi.org/10.1007/s12035-022-03011-1

Article  CAS  PubMed  Google Scholar 

Costello DJ, Kilbride RD, Cole AJ (2009) Cryptogenic new onset refractory status epilepticus (NORSE) in adults-infectious or not? J Neurol Sci 277(1-2):26–31. https://doi.org/10.1016/j.jns.2008.10.007

Article  PubMed  Google Scholar 

Hanin A, Cespedes J, Dorgham K, Pulluru Y, Gopaul M, Gorochov G, Hafler DA, Navarro V, Gaspard N, Hirsch LJ (2023) Cytokines in new-onset refractory status epilepticus predict outcomes. Ann Neurol 94(1):75–90. https://doi.org/10.1002/ana.26627

Article  CAS  PubMed  Google Scholar 

Wickstrom R, Taraschenko O, Dilena R, Payne ET, Specchio N, Nabbout R, Koh S, Gaspard N, Hirsch LJ, International NCG (2022a) International consensus recommendations for management of new onset refractory status epilepticus (NORSE) incl. febrile infection-related epilepsy syndrome (FIRES): statements and supporting evidence. Epilepsia 63(11):2840–2864. https://doi.org/10.1111/epi.17397

Article  PubMed  PubMed Central  Google Scholar 

Vezzani A, French J, Bartfai T, Baram TZ (2011) The role of inflammation in epilepsy. Nat Rev Neurol 7(1):31–40. https://doi.org/10.1038/nrneurol.2010.178

Article  CAS  PubMed  Google Scholar 

Nabbout R, Vezzani A, Dulac O, Chiron C (2011) Acute encephalopathy with inflammation-mediated status epilepticus. Lancet Neurol 10(1):99–108. https://doi.org/10.1016/S1474-4422(10)70214-3

Article  PubMed  Google Scholar 

Wickstrom R, Taraschenko O, Dilena R, Payne ET, Specchio N, Nabbout R, Koh S, Gaspard N, Hirsch LJ, International NCG (2022b) International consensus recommendations for management of new onset refractory status epilepticus (NORSE) including febrile infection-related epilepsy syndrome (FIRES): summary and clinical tools. Epilepsia 63(11):2827–2839. https://doi.org/10.1111/epi.17391

Article  PubMed  PubMed Central  Google Scholar 

Sheikh Z, Hirsch LJ (2023) A practical approach to in-hospital management of new-onset refractory status epilepticus/febrile infection related epilepsy syndrome. Front Neurol 14:1150496. https://doi.org/10.3389/fneur.2023.1150496

Article  PubMed  PubMed Central  Google Scholar 

Vinette SA, Young GB, Khosravani H (2023) Early identification of NORSE and transfer to care setting with appropriate supports: a proposed algorithm. Front Neurol 14:1072020. https://doi.org/10.3389/fneur.2023.1072020

Article  PubMed  PubMed Central  Google Scholar 

Khawaja AM, DeWolfe JL, Miller DW, Szaflarski JP (2015) New-onset refractory status epilepticus (NORSE)—The potential role for immunotherapy. Epilepsy Behav 47:17–23. https://doi.org/10.1016/j.yebeh.2015.04.054

Article  PubMed  Google Scholar 

Cabrera Kang CM, Gaspard N, LaRoche SM, Foreman B (2017) Survey of the diagnostic and therapeutic approach to new-onset refractory status epilepticus. Seizure 46:24–30. https://doi.org/10.1016/j.seizure.2017.02.003

Article  PubMed  Google Scholar 

Yanagida A, Kanazawa N, Kaneko J, Kaneko A, Iwase R, Suga H, Nonoda Y, Onozawa Y, Kitamura E, Nishiyama K, Iizuka T (2020) Clinically based score predicting cryptogenic NORSE at the early stage of status epilepticus. Neurol Neuroimmunol Neuroinflamm. https://doi.org/10.1212/NXI.0000000000000849

Article  PubMed  PubMed Central  Google Scholar 

Chinnery PF (2015) Mitochondrial disease in adults: what’s old and what’s new? EMBO Mol Med 7(12):1503–1512. https://doi.org/10.15252/emmm.201505079

Article  CAS  PubMed  PubMed Central  Google Scholar 

Kim HJ, Lee SA, Kim HW, Kim SJ, Jeon SB, Koo YS (2020) The timelines of MRI findings related to outcomes in adult patients with new-onset refractory status epilepticus. Epilepsia 61(8):1735–1748. https://doi.org/10.1111/epi.16620

Article  PubMed  Google Scholar 

Schoeler NE, Simpson Z, Zhou R, Pujar S, Eltze C, Cross JH (2021) Dietary management of children with super-refractory status epilepticus: a systematic review and experience in a single UK tertiary centre. Front Neurol 12:643105. https://doi.org/10.3389/fneur.2021.643105

Article  PubMed  PubMed Central  Google Scholar 

Dozieres-Puyravel B, Hohn S, Auvin S (2021) Considering safety and patient tolerance in the use of ketogenic diet in the management of refractory and super-refractory status epilepticus: a systematic review. Expert Rev Neurother 21(11):1303–1308. https://doi.org/10.1080/14737175.2021.1956905

Article  CAS  PubMed  Google Scholar 

Horino A, Kuki I, Inoue T, Nukui M, Okazaki S, Kawawaki H, Togawa M, Amo K, Ishikawa J, Ujiro A, Shiomi M, Sakuma H (2021) Intrathecal dexamethasone therapy for febrile infection-related epilepsy syndrome. Ann Clin Transl Neurol 8(3):645–655. https://doi.org/10.1002/acn3.51308

Article  CAS  PubMed  PubMed Central  Google Scholar 

Stavropoulos I, Khaw JH, Valentin A (2023) Neuromodulation in new-onset refractory status epilepticus. Front Neurol 14:1195844. https://doi.org/10.3389/fneur.2023.1195844

Article