References for the scientific symposium curated by James Riviello, MD.Recognition NORSE/FIRES:
Haanpaa A, Laakso SM, Kinnunen A, Kamppi L, Forss N. Early clinical features of new-onset refractory status epilepticus. BMC Neurology:2022;22:495.
Iizuka T, Kanazawa N, Kaneko J, et al. Cryptogenic NORSE: Its distinctive clinical features and response to immunotherapy. Neurol Neuroimmunol Neuroinflamm 2017;4;3396;doi: 10.1212/NXI.00000000000396.
Lattanzi S, Leitinger M, Rocchi C, et al. Unraveling the enigma of new-onset refractory status epilepticus: A systematic review of aetiologies. Eur J Neurol 2022;29:626-647.
Koh S, Wirrell E, Vezzani A, Nabbout R; Muscal E ; Kaliakatsos M; Wickström R, Riviello J, Brunklaus A, Payne E, Valentin A, Wells E, Carpenter J, Lee K, Lai YC, Eschbach K, Press C, Gorman M, Coral S, Roche W, Mangum T. Proposal to Optimize Evaluation and Treatment of Febrile Infection-Related Epilepsy Syndrome (FIRES): A Report from FIRES Workshop. Epilepsia Open 2021;6:62-72.
Wickstrom R, Taraschenko O, Dilena R, Payne ET, Specchio N, Nabbout R, Koh S, Gaspard N, Hirsch LJ; International NORSE Consensus Group. International consensus recommendations for management of New Onset Refractory Status Epilepticus (NORSE) including Febrile Infection-Related Epilepsy Syndrome (FIRES): Summary and Clinical Tools. Epilepsia 2022; 63:2827-2839.
Wickstrom R, Taraschenko O, Dilena R, Payne ET, Specchio N, Nabbout R, Koh S, Gaspard N, Hirsch LJ; International NORSE Consensus Group. International consensus recommendations for management of New Onset Refractory Status Epilepticus (NORSE) including Febrile Infection-Related Epilepsy Syndrome (FIRES): Statements and supporting evidence. Epilepsia 2022;63:2840-2864.
Yanigida A, Kanazawa N, Kaneko J, et al. Clinically based score predicting cryptogenic NORSE at the early stage of status epilepticus. Neurol Neuroimmunol Neuroinflamm 2022;7;e849. Doi: 10.1212/NXI.00000000000849.
Biomarkers for NORSE/FIRES:
Culleton S, Talenti G, Kaliakatsos. The spectrum of neuroimaging findings in febrile infection-related epilepsy syndrome (FIRES): A literature review. Epilepsia 2019;60:585-592.
Farias-Moeller R, Bartolini L, Staso K, Schreiber JM, Carpenter JL. Early ictal and interictal patterns in FIRES: The sparks before the blaze. Epilepsia 2017;58:1340-1348.
Hanin A, Cespedes J, Dorgham, et al. Cytokines in new-onset refractory status epilepticus predict outcomes. Ann Neurol 2023:94:75-90.
Koepp M, Arstad E, Bankstahl JP, Neuroinflammation imaging markers for epileptogensis. Epilepsia 2017;58(Suppl. 3):11-19.
Lai YC, Abou-El-Kheir G, Nguyen T, Hanerhoff M, Riviello JJ, Muscal E. Systemic inflammatory markers and EEG features of children with FIRES receiving anakinra. Ann Clin Transl Neurol 2023;10:440-446.
Melletti S, Slonkova J, Mareckova I, et al. Claustrum damage and refractory status epilepticus following febrile illness. Neurology 2015;85:1224-1232.
Stredny C, Rotenberg A, Leviton A, Loddenkemper T. Systemic inflammation as a biomarker of seizure propensity and a target for treatment to reduce seizure propensity. Epilepsia Open 2023;8:221-234.
Mechanisms Refractory Status Epilepticus and Treatments:
Buratti S, Giacheri E, Palmieri A, et al. Ketamine as advanced second-line treatment in benzodiazepine-refractory convulsive status epilepticus in children. Epilepsia 2023;64:797-810.
Farias-Moeller R, Bartolini L, Pasupuleti A, et al. A practical approach to ketogenic diet in the pediatric intensive care unit fir super-refractory status epilepticus/ Neurocrit Care 2017;26:267-272.
Ho CJ, Lin CH, Lu YT, et al. Perampanel treatment for refractory status epilepticus in a neurological intensive care unit. Neurocrit Care 2019;31:24-29.
Nabbout R, Mazzuca M, Hubert P, et al. Efficacy of ketogenic diet in severe refractory status epilepticus initiating fever induced refractory epileptic encephalopathy in school age children (FIRES). Epilepsia 2010;51:2033-2037.
Nabbout R, Matricardi S, De Liso P et al. Ketogenic diet for super-refractory status epilepticus (SRSE) with NORSE and FIRES: Single tertiary center experience and literature data. Frontiers Neuro 2023 Apr 13 ;14 :1134827. Doi : 10.3389/fneur.2023. 1134827. Ecollection 2023.
Naylor DE. In the fast lane: Receptor trafficking during status epilepticus. Epilepsia Open 2023;8(Suppl. 1):S35-65.
Nilo A, Vogrig A, Belluzzo M, et al. Efficacy of perampanel in refractory and super-refractory status epilepticus with suspected inflammatory etiology: A case series. Pharmaceuticals 2024;17:28.
Niquet J, Baldwin R, Suchomelova L, et al. Treatment of experimental status epilepticus with synergistic drug combinations. Epilepsia 2017;58:e49-e53.
Niquet J, Lumley L, Baldwin R, et al. Early polytherapy for benzodiazepine-refractory status epilepticus. Epilepsy and Behavior 2019;101:106367.
Sankar R. Treatment of status epilepticus: Physiology, pharmacology, and future directions. Epilepsy Open 2023;8(Suppl. 1):S141-S148.
Souidan H, Machado RA, Lagnf A, Elsayed M. Double antiglutamatergic treatment in patients with status epilepticus: A case series. Epilepsy and Behavior 2022;137:108954.
Promising Immunotherapies:
Farias-Moeller R, Hanin A, Ahsan S, et al. Intrathecal dexamethasone as a FIRES extinguisher: A twelve patient clinical experience with usage of intrathecal dexamethasone for Febrile-Infection Related Epilepsy Syndrome. Neurocrit Care: https://doi.org/10.1007/s12028-025-02254-9
Fisher KS, Ankar A, Cokley J, Muscal E, Riviello JJ, Lai YC. Combined systemic immunotherapy and intrathecal dexamethasone in febrile infection related epilepsy syndrome. Ann Clin Transl Neurol 2025;Feb 11.doi: 10.1002/acn3.70011. Online ahead of print.
Hanin A, Muscal E, Hirsch LJ. Second-line immunotherapy in new onset refractory status epilepticus. Epilepsia 2024:65:1203-1223.
Horino A, Kuki I, Inoue T, et al. Intrathecal dexamethasone therapy for febrile infection-related epilepsy syndrome. Ann Clin Transl Neuro 202;8:645-655.
Jang Y, Lee WJ, Lee HS et al. Tofacitinib treatment for refractory status epilepticus. Epilepsia 2021;62:e53-e59.
Lai YC, Muscal E, Wells E, Shukla N, Eschbach K, Lee KH, Kaliakatsos M, Desai N, Wickstrom R, Viri M, Freri E, Granata T, Nangia S, Dilena R, Brunklaus A, Wainwright MS, Gorman MP, Stredny CM, Asiri A, Hundallah K, Doja A, Payne E, Wirrell E, Koh S, Carpenter JL, Riviello J. Anakinra Usage in Febrile Infection Related Epilepsy Syndrome (FIRES): An International Retrospective Cohort. Ann Clin Transl Neurol 2020;7:2467-2474.
Mehta NP, Sawdy R, Maloney K, intrathecal dexamethasone on febrile-illness related epilepsy syndrome. Neurol Clin Pract 2023;13:e200153
Sun H, Ma D, Cheng Y, et al. The JAK-STAT signaling pathway in epilepsy. Current Neuropharmacology 2023;21:2049-2069.
Tipton AE, Cruz Del Angel Y, Hixson K, et al. Selective neuronal knockout of STAT 3 function inhibits epilepsy progression, improves cognition, and restores dysregulated gene networks in a temporal lobe epilepsy model. Ann Neurol 2023;94:106-122.
Yarmini JM, Sandweiss AJ, Salazar KP, et al. IT-DEX and B cell depletion in a child with anti-GAD 65 autoimmune encephalitis presenting as NORSE: A case report. J Immunology 2024;395:578430. Doi: 10.1016/j.jneuroim.2024.578430. Epub 2024 Aug 8.
Prognosis/Communication of NORSE:
Eschbach K, Reedy J. Gofton T, et al. Navigating life after new-onset refractory status epilepticus (NORSE) and febrile-illness related epilepsy syndrome (FIRES): Insights from caregiver and patient interviews. Ep Behav 2025 Feb:163:110236. Doi: 10.1016/j.yebeh.2024.110236. Epub 2024 Dec 31.
Farias-Moeller R, Wood A, Sawdy et al. Parental perception of FIRES outcomes, emotional stats and social media usage. Epilepsia Open 2021;6:539-547.
Farias-Moeller R, Wong N. Supporting parents while their child is receiving neurocritical care. Semin Pediatr Neurol 2024. 2024 Apr;49:101116. Doi: 10.1016/j.spen.2024.101116. Epub 2024 Jan 21.
Kazazian K, Gaspard N, Hirsch LJ, et al. Communication trends over time in new-onset refractory status epilepticus (NORSE); Interim analysis from the NORSE/FIRES family registry. Ep Behav 2024 Nov;160:110023, doi: 10.1016/j.yebeh.2024.110023. Epub 2024 Sep 5.
Biorepository:
Hanin A, Cespedes J, Pulluru Y, et al. Review and standard operating procedures for collection of biospecimens and analysis of biomarkers in new onset refractory status epilepticus. Epilepsia 2023;64:1444-1457.
Kazazian K, Gaspard N, Hirsch LJ, et al. Age-associated differences in FIRES: Characterizing prodromal presentation and long-term outcomes via the web-based NORSE/FIRES family registry. Epilepsia 2025;66:e35-e40.
