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NORSE‑ICM

Paris Brain Institute

NORSE / FIRES Bibliography

Compiled list of published case series, case reports and reviews related to NORSE and FIRES, 2001–2026.

150

Case series

184

Case reports

62

Reviews

29

Pathophysiology studies

Case Series

150 published case series — 2001 to 2026

2001–2005Case Series (2001 – 2005)5 articles
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  1. Sahin et al. Outcome of severe refractory status epilepticus in children. 2001. Case series. PMID 11879350
  2. Van Lierde et al. De novo cryptogenic refractory multifocal febrile status epilepticus in young adults. 2003. PMID 12892002
  3. Baxter et al. Idiopathic catastrophic epileptic encephalopathy with acute onset intractable status. 2003. PMID 12915084
  4. Kramer et al. Severe refractory status epilepticus owing to presumed encephalitis. 2005. PMID 15832606
  5. Wilder‑Smith et al. The NORSE syndrome: defining a disease entity. 2005. PMID 16123813
2006–2010Case Series (2006 – 2010)12 articles
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  1. Mikaeloff et al. Devastating epileptic encephalopathy in school‑aged children (DESC): a pseudo encephalitis. 2006. PMID 16469483
  2. Saito et al. Acute encephalitis with refractory, repetitive partial seizures: case reports of this unusual post‑encephalitic epilepsy. 2007. PMID 17008042
  3. Lin et al. Analysis of status epilepticus related presumed encephalitis in children. 2008. PMID 17584506
  4. Shyu et al. Acute encephalitis with refractory, repetitive partial seizures. 2008. PMID 18078728
  5. Glaser et al. Refractory status epilepticus in suspect encephalitis. 2008. PMID 18097641
  6. Costello et al. Cryptogenic new‑onset refractory status epilepticus in adults — infectious or not? 2009. PMID 19013586
  7. Specchio et al. Epileptic encephalopathy in children possibly related to immune‑mediated pathogenesis. 2010. PMID 19850427
  8. Sakuma et al. Acute encephalitis with refractory, repetitive partial seizures (AERRPS): a peculiar form of childhood encephalitis. 2010. PMID 20028339
  9. Van Baalen et al. Febrile infection‑related epilepsy syndrome (FIRES): a non‑encephalitic encephalopathy in childhood. 2010. PMID 20345937
  10. Nabbout et al. Efficacy of ketogenic diet in severe refractory status epilepticus initiating fever‑induced refractory epileptic encephalopathy in school‑age children (FIRES). 2010. PMID 20813015
  11. Okumura et al. Amplitude-integrated electroencephalography in patients with acute encephalopathy with refractory, repetitive partial seizures. 2010. PMID 20092972
  12. Fugate et al. Prolonged high-dose isoflurane for refractory status epilepticus: is it safe?. 2010. PMID 20841413
2011–2015Case Series (2011 – 2015)16 articles
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  1. Mazzuca et al. 18F‑FDG PET reveals frontotemporal dysfunction in children with fever‑induced refractory epileptic encephalopathy. 2011. PMID 21149491
  2. Kramer et al. Febrile infection‑related epilepsy syndrome (FIRES): pathogenesis, treatment, and outcome — a multicenter study on 77 children. 2011. PMID 21883180
  3. Kramer et al. Febrile infection‑related epilepsy syndrome (FIRES): does duration of anesthesia affect outcome? 2011. PMID 21967356
  4. Nam et al. The role of ketogenic diet in the treatment of refractory status epilepticus. 2011. PMID 22003821
  5. Howell et al. Long‑term follow‑up of febrile infection‑related epilepsy syndrome. 2012. PMID 22191582
  6. Lin et al. Therapeutic hypothermia for febrile infection‑related epilepsy syndrome in two patients. 2012. PMID 23127267
  7. Li et al. Plasma exchange in cryptogenic new‑onset refractory status epilepticus. 2013. PMID 23068971
  8. Gall et al. Five cases of new‑onset refractory status epilepticus (NORSE) syndrome: outcomes with early immunotherapy. 2013. PMID 23333762
  9. Caraballo et al. Febrile infection‑related epilepsy syndrome: a study of 12 patients. 2013. PMID 23643626
  10. Sort et al. Ketogenic diet in three cases of childhood refractory status epilepticus. 2013. PMID 23751291
  11. Caraballo et al. Ketogenic diet in pediatric patients with refractory focal status epilepticus. 2014. PMID 25454503
  12. Singh et al. Cognitive outcomes in febrile infection‑related epilepsy syndrome treated with the ketogenic diet. 2014. PMID 25332495
  13. Ueda et al. Effect of levetiracetam in acute encephalitis with refractory repetitive partial seizures during acute and chronic phase. 2015. PMID 25174548
  14. Khawaja et al. New‑onset refractory status epilepticus (NORSE) — the potential role for immunotherapy. 2015. PMID 26010959
  15. Gaspard et al. New‑onset refractory status epilepticus: etiology, clinical features and outcome. 2015. PMID 26296517
  16. Tan et al. Use of magnesium sulfate infusion for the management of febrile illness‑related epilepsy syndrome: a case series. 2015. PMID 28503584
2016–2020Case Series (2016 – 2020)27 articles
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  1. Rivas‑Coppola et al. Chronological evolution of MRI findings in children with febrile infection‑related epilepsy syndrome (FIRES). 2016. PMID 26597039
  2. Patil et al. Clinical profile and treatment outcome of febrile infection‑related epilepsy syndrome in South Indian children. 2016. PMID 27293328
  3. Appavu et al. Ketogenic diet treatment for pediatric super‑refractory status epilepticus. 2016. PMID 27475280
  4. Farias Moeller et al. A practical approach to ketogenic diet in the pediatric intensive care unit for super‑refractory status epilepticus. 2017. PMID 27553113
  5. Gofshteyn et al. Cannabidiol as a potential treatment for febrile infection‑related epilepsy syndrome (FIRES) in the acute and chronic phases. 2017. PMID 27655472
  6. Meletti et al. New‑onset refractory status epilepticus with claustrum damage: definition of clinical and neuroimaging features. 2017. PMID 28396650
  7. Farias Moeller et al. Early ictal and interictal patterns in FIRES: the sparks before the blaze. 2017. PMID 28555777
  8. Iizuka et al. Cryptogenic NORSE: its distinctive clinical features and response to immunotherapy. 2017. PMID 28959704
  9. Jafarpour et al. New‑onset status epilepticus in pediatric patients: causes, characteristics and outcomes. 2018. PMID 29373157
  10. Lee et al. Febrile infection‑related epilepsy syndrome (FIRES): therapeutic complications, long‑term neurological and neuroimaging follow‑up. 2018. PMID 29453111
  11. Farias Moeller et al. Fueling the FIRES: hemophagocytic lymphohistiocytosis in FIRES. 2018. PMID 30132834
  12. Jun et al. Tocilizumab treatment for new‑onset refractory status epilepticus. 2018. PMID 30408233
  13. Strohm et al. FDG‑PET and MRI in the evolution of new‑onset refractory status epilepticus. 2019. PMID 30679215
  14. Peng et al. Ketogenic diet as treatment for super‑refractory status epilepticus in FIRES. 2019. PMID 31105638
  15. Sa et al. Centromedian thalamic nuclei deep brain stimulation and anakinra treatment for FIRES — two different outcomes. 2019. PMID 31446001
  16. Choi et al. Prognostic significance of subsequent extra‑temporal involvement in cryptogenic NORSE initially diagnosed with limbic encephalitis. 2019. PMID 31669912
  17. Lam et al. Short‑term and long‑term outcome of FIRES in children. 2020. PMID 31035103
  18. Husari et al. New‑onset refractory status epilepticus in children: etiologies, treatments and outcomes. 2020. PMID 31568262
  19. Aurangzeb et al. New‑onset super‑refractory status epilepticus: a case series. 2020. PMID 31757748
  20. Gugger et al. New‑onset refractory status epilepticus: a retrospective cohort study. 2020. PMID 31830676
  21. Cantarin‑Extremera et al. Tocilizumab in pediatric refractory status epilepticus and acute epilepsy: experience in two patients. 2020. PMID 31935626
  22. Wang et al. Is rat hippocampus section immunostaining an indicator for immunotherapy in cryptogenic adult NORSE? 2020. PMID 32066091
  23. Kim et al. Timelines of MRI findings related to outcomes in adult NORSE. 2020. PMID 32715470
  24. Yanagida et al. Clinically based score predicting cryptogenic NORSE at early stage of status epilepticus. 2020. PMID 32727813
  25. Matthews et al. New‑onset super‑refractory status epilepticus: a case series of 26 patients. 2020. PMID 32943479
  26. Lai et al. Anakinra usage in FIRES: an international cohort. 2020. PMID 33506622
  27. Wang et al. The ketogenic diet for paediatric patients with super‑refractory status epilepticus in FIRES. 2020. PMCID s42494‑020‑00013‑1
2021–2023Case Series (2021 – 2023)26 articles
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  1. Horino et al. Intrathecal dexamethasone therapy for febrile infection‑related epilepsy syndrome. 2021. PMID 33547757
  2. Manganotti et al. Intravenous immunoglobulin response in new‑onset refractory status epilepticus (NORSE) COVID‑19 adult patients. 2021. PMID 33709220
  3. Sculier et al. Clinical presentation of new‑onset refractory status epilepticus in children (the pSERG cohort). 2021. PMID 34091885
  4. Nass et al. Permanent loss of independence in adult FIRES survivors: an underestimated and unsolved challenge. 2021. PMID 34091969
  5. Yildirim et al. Therapeutic plasma exchange in clinical pediatric neurology practice: experience from a tertiary referral hospital. 2021. PMID 34304066
  6. Suchdev et al. Histopathology of new‑onset refractory status epilepticus (NORSE) in adults. 2021. PMID 34740145
  7. Neligan et al. The Queen Square Neuro‑ICU experience in new‑onset refractory status epilepticus (NORSE). 2021. PMID 34781063
  8. Wu et al. A retrospective study of 92 children with new‑onset refractory status epilepticus. 2021. PMID 34794014
  9. Lim et al. Utility and safety of perampanel in pediatric FIRES and other drug‑resistant epilepsies. 2021. PMID 34820471
  10. Nausch et al. NORSE and FIRES of unknown aetiology: a comparison of the incomparable? 2022. PMID 35042004
  11. Aledo‑Serrano et al. Anakinra and tocilizumab in the chronic phase of FIRES: effectiveness and safety from a case series. 2022. PMID 35759951
  12. Jain et al. Enteral lorazepam as a promising weaning strategy for midazolam‑responsive FIRES: a case series. 2022. PMID 35770765
  13. Werbaneth et al. A retrospective cohort study of NORSE: clinical features, timing of immunotherapy and outcomes. 2022. PMID 35892128
  14. Espino et al. Long‑term outcomes after NORSE treated with vagus nerve stimulation. 2022. PMID 36177520
  15. Pavone et al. FIRES: a severe encephalopathy with status epilepticus; literature review and two new cases. 2022. PMID 36527084
  16. Tainski de Azevedo et al. New‑onset refractory status epilepticus in paediatric patients: causes, characteristics and outcomes. 2022. PMID 36530417
  17. Haanpää et al. Early clinical features of new‑onset refractory status epilepticus (NORSE) in adults. 2022. PMID 36539824
  18. Lai et al. Systemic inflammatory markers and EEG features of children with FIRES receiving anakinra. 2023. PMID 36645080
  19. Shi et al. Long‑term outcomes of adult cryptogenic FIRES. 2023. PMID 36686522
  20. Girardin et al. Treatment of new‑onset refractory status epilepticus and FIRES with tocilizumab in a child and a young adult. 2023. PMID 36961094
  21. Shrestha et al. Long‑term neuropsychological outcomes in children with FIRES treated with anakinra. 2023. PMID 36970506
  22. Fetta et al. Cannabidiol in the acute phase of FIRES. 2023. PMID 37042946
  23. Nabbout et al. Ketogenic diet for super‑refractory status epilepticus (SRSE) with NORSE and FIRES. 2023. PMID 37122314
  24. Soler Wenglein et al. No evidence of neuronal/glial autoantibodies in FIRES: a prospective clinic‑serologic analysis. 2023. PMID 37599999
  25. Eisele et al. The role of neuronal antibodies in cryptogenic new‑onset refractory status epilepticus. 2023. PMID 37607299
  26. Furlanis et al. Role of vagus nerve stimulation in refractory and super‑refractory status epilepticus: a pediatric case series. 2023. PMID 38002548
2024–2026Case Series (2024 – 2026)64 articles
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  1. Moreno‑Braurer P et al. Spectrum, evolution and clinical relationship of MRI in 31 children with FIRES. 2024. PMID 37798920
  2. Alaskar et al. Time to FIRE NORSE: a single acronym for heterogeneous presentation. 2024. PMID 38330780
  3. Bai et al. FIRES with claustrum lesion: an underdiagnosed inflammatory encephalopathy. 2024. PMID 38342839
  4. Stretti et al. Long‑term outcome in new‑onset refractory status epilepticus: a retrospective study. 2024. PMID 38475798
  5. Costello et al. Clinical outcomes among initial survivors of cryptogenic new‑onset refractory status epilepticus (NORSE). 2024. PMID 38498313
  6. Jimenez et al. Comparative analysis of patients with FIRES versus without prior fever: interim analysis. 2024. PMID 38625055
  7. Oliger et al. Responsive neurostimulation as therapy for epilepsy following NORSE: case series and literature review. 2024. PMID 38640819
  8. Hanin et al. Trends in management of NORSE patients from 2016 to 2023: an interim analysis. 2024. PMID 38837761
  9. Jang et al. Prognosis prediction and immunotherapy optimisation for cryptogenic NORSE. 2024. PMID 39237150
  10. Reecher et al. New‑onset refractory status epilepticus with diffuse cerebral restricted diffusion in young children. 2025. PMID 39541921
  11. Yorichika et al. Early initiation of intravenous cyclophosphamide and one‑year outcomes in super‑refractory cryptogenic NORSE. 2025. PMID 39701580
  12. Nemati et al. NORSE and FIRES cases in children: a retrospective cohort study in South of Iran. 2025. PMID 39896696
  13. Bandi et al. Challenges in management of FIRES: real‑world experience from a large paediatric cohort. 2025. PMID 39908710
  14. Fisher et al. Combined systemic immunotherapy and intrathecal dexamethasone in FIRES. 2025. PMID 39932904
  15. Saad et al. FIRES in children: a retrospective cohort study. 2025. PMID 39988600
  16. Liu et al. Vagus nerve stimulation and fast parameter programming protocol in children with FIRES in ICU. 2025. PMID 40000337
  17. Adiga et al. Phenotype, outcome, interleukins and miRNA levels in NORSE: a prospective cohort study. 2025. PMID 40047557
  18. Goh et al. Clinical outcomes of cryptogenic new‑onset refractory status epilepticus in Singapore. 2025. PMID 40053179
  19. Pasqualini et al. Dramatic response to delayed tocilizumab treatment in new‑onset refractory status epilepticus. 2025. PMID 40100558
  20. Diao et al. Clinical analysis of seven cases of cryptogenic NORSE. 2025. PMID 40193644
  21. Halawani et al. Comparative analysis of NORSE in adult and pediatric patients. 2025. PMID 40251332
  22. Farias Moeller et al. Intrathecal dexamethasone as a FIRES extinguisher: experience in 12 patients. 2025. PMID 40279013
  23. Spoden et al. A case series and review of FIRES. 2025. PMID 40310164
  24. Ferreira et al. Performance of cryptogenic NORSE score in a Brazilian cohort. 2025. PMID 40318023
  25. Chin et al. Pediatric new‑onset super‑refractory status epilepticus (NOSRSE): case series. 2025. PMID 40318399
  26. Muccioli et al. Secondary sclerosing cholangitis in critically ill patients with FIRES. 2025. PMID 40343187
  27. Teo et al. Comparative clinical outcomes in children with FIRES. 2025. PMID 40382999
  28. Kilmer et al. Role of metabolic testing in adult NORSE: single‑centre retrospective study. 2025. PMID 40470573
  29. Feng et al. Minocycline in chronic management of FIRES: case series and literature review. 2025. PMID 40481521
  30. Zhao et al. Clinical and neuroimaging features of patients with claustrum sign. 2025. PMID 40496135
  31. He et al. Observational study of tocilizumab in children with FIRES. 2025. PMID 40556126
  32. Shin et al. Analysis of early EEG changes after tocilizumab treatment in NORSE. 2025. PMID 40563808
  33. Giussani et al. Retrospective multicenter study on cryptogenic NORSE/FIRES treated with anakinra. 2025. PMID 40614647
  34. Wang et al. Clinical characteristics of 27 children with FIRES in a single center. 2025. PMID 40625895
  35. Shrestha et al. Gastrointestinal symptoms are common in patients with NORSE. 2025. PMID 40835780
  36. Wang et al. Vagus nerve stimulation combined with ketogenic diet versus ketogenic diet alone in children with FIRES. 2025. PMID 41047417
  37. Das et al. Spectrum of pediatric acute leukoencephalopathy with restricted diffusion presenting as FIRES. 2025. PMID 41129842
  38. Peng et al. Factors associated with long‑term outcomes in people with new‑onset refractory epilepticus. 2026. PMID 41277767
  39. Han et al. Symmetrical claustrum sign in pediatric FIRES: diagnostic value and clinical implications. 2025. PMID 41427492
  40. Fan et al. Ketogenic diet therapy for adult FIRES: case series of five patients and efficacy analysis. 2025. PMID 41466766
  41. Lattanzi et al. Predicting epilepsy after NORSE due to autoimmune encephalitis: the DAME score. 2025. PMID 41467927
  42. Levi Strauss et al. Extreme type of new‑onset refractory status epilepticus with stimulus‑induced seizures. 2026. PMID 40847775
  43. Gruen et al. Quality of life over time after new‑onset refractory status epilepticus. 2026. PMID 40944696
  44. Murugesu et al. Tocilizumab for super‑refractory status epilepticus in children with FIRES: case series. 2026. PMID 41401655
  45. Lim et al. New‑onset refractory status epilepticus associated with Sjögren's disease. 2026. PMID 41561677
  46. Yan et al. Efficacy differences between tocilizumab and ketogenic diet during acute phase of FIRES in children. 2026. PMID 41633319
  47. Chidambaram et al. Pediatric new‑onset super‑refractory status epilepticus: potential of timely immunotherapy in a resource‑limited PICU. 2026. PMID 41679100
  48. Li et al. Metabolism‑corrected propofol exposure intensity and long‑term IQ in pediatric FIRES: retrospective cohort study. 2026. PMID 41767518
  49. Yoo et al. Vagus nerve stimulation as adjunct therapy for super‑refractory status epilepticus including NORSE. 2026. PMID 41875755
  50. Deng et al. Clinical outcomes of tocilizumab usage in cryptogenic FIRES at acute and chronic phase. 2026. PMID 42094697
  51. Jayalakshmi et al. Clinical characteristics, etiology, and outcome of NORSE in adults. 2026. PMID 41498863
  52. Abe et al. Longitudinal changes in brain volume, cognitive function and seizure outcomes in cryptogenic NORSE. 2026. PMID 42184585
  53. Tian et al. The electroencephalographic patterns in the acute phase of febrile infection related epilepsy syndrome in children. 2026. PMID 42241938
  54. Pang et al. Case report: tocilizumab in pediatric febrile infection-related epilepsy syndrome. 2026. PMID 42317346
  55. Gilani et al. Acute and Long-Term EEG and seizure characteristics in new onset refractory status epilepticus (NORSE). 2025. PMID 42359767
  56. Romozzi et al. Cenobamate use in super-refractory status epilepticus: A report of three cases. 2025. PMID 42400400
  57. Soler Wenglein et al. Febrile infection-related epilepsy syndrome (FIRES): duration of therapeutic coma and treatment intensity correlate with poorer functional outcome. 2026. PMID 42537571
  58. Hawksworth et al. New onset refractory status epilepticus (NORSE) versus refractory status epilepticus not meeting NORSE criteria: A comparative clinical and electroencephalography-based study. 2026. PMID 42446501
  59. Lin et al. Multidisciplinary management and nursing care for pediatric patients with febrile infection-related epilepsy syndrome in the acute phase: A case series. 2026. PMID 42470032
  60. Cruz-Perez et al. Management Challenges and Clinical Characteristics of Refractory Status Epilepticus in Mexico: A 102 Patient Retrospective Cohort Study. 2026. PMID 42568019
  61. Khan et al. Claustrum Sign in Febrile Infection-Related Epilepsy Syndrome. 2026. PMID 42619283
  62. Han et al. Electroencephalographic and neuroimaging prognosticators in pediatric FIRES with claustrum lesions: A 21-case retrospective study. 2026. PMID 42641489
  63. Junemann et al. Clinical outcomes and unmet therapeutic needs in new-onset refractory status epilepticus: evidence from a retrospective cohort. 2026. PMID 42665853
  64. Liu et al. Efficacy and safety of tocilizumab in the chronic phase of febrile infection-related epilepsy syndrome: a retrospective cohort study. 2026. PMID 42696242

Case Reports

184 published case reports — 2006 to 2026

2006–2010Case Reports (2006 – 2010)2 articles
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  1. Okanishi et al. Refractory epilepsy accompanying acute encephalitis with multifocal cortical lesions: possible autoimmune etiology. 2007. PMID 17442514
  2. Boyd et al. New‑onset refractory status epilepticus with restricted DWI and neuronophagia in the pulvinar. 2010. PMID 20308685
2011–2015Case Reports (2011 – 2015)23 articles
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  1. Milh et al. New‑onset refractory convulsive status epilepticus associated with serum neuropil auto‑antibodies in a school‑aged child. 2011. PMID 21075572
  2. Illingworth et al. Elevated VGKC‑complex antibodies in a boy with fever‑induced refractory epileptic encephalopathy in school‑age children (FIRES). 2011. PMID 21592118
  3. Specchio et al. Acute‑onset epilepsy triggered by fever mimicking FIRES: the role of PCDH19 gene mutation. 2011. PMID 21777234
  4. Ismail et al. AERRPS, DESC, NORSE, FIRES: multi‑labeling or distinct epileptic entities? 2011. PMID 22004046
  5. Boyd et al. Temporal lobe epilepsy after refractory status epilepticus: an illustrative case and review of the literature. 2012. PMID 22953058
  6. Wakamoto et al. An immunologic case study of acute encephalitis with refractory, repetitive partial seizures. 2012. PMID 22265641
  7. Nozaki et al. Reversible splenic lesion in a patient with febrile infection‑related epilepsy syndrome (FIRES). 2013. PMID 23794447
  8. Verma et al. New‑onset refractory status epilepticus (NORSE) as the heralding manifestation of herpes simplex encephalitis. 2013. PMID 23887985
  9. Puoti et al. New‑onset refractory status epilepticus mimicking herpes virus encephalitis. 2013. PMID 24163672
  10. Pankajakshan et al. Refractory status epilepticus complicated by drug‑induced involuntary movements. 2014. PMID 24518529
  11. Barros et al. Resective surgery in the treatment of super‑refractory partial status epilepticus secondary to NMDAR antibody encephalitis. 2014. PMID 24594428
  12. Gedik et al. Therapeutic plasma exchange for malignant refractory status epilepticus: a case report. 2014. PMID 24630284
  13. Matsuzono et al. Ketogenic diet therapy is effective in encephalitis with refractory seizures. 2014. PMID 24735348
  14. Nolan et al. Anti‑N‑Methyl‑D‑Aspartate receptor encephalitis presenting to the emergency department with status epilepticus. 2014. PMID 25227654
  15. Ibrahim et al. New‑onset refractory status epilepticus in a young man with H1N1 infection. 2014. PMID 25379313
  16. Hainsworth et al. Treatment‑responsive GABA(B)‑receptor limbic encephalitis presenting as new‑onset super‑refractory status epilepticus (NORSE) in a deployed soldier. 2014. PMID 25465280
  17. Byler et al. Rapid onset of hippocampal atrophy in febrile infection‑related epilepsy syndrome (FIRES). 2014. PMID 23400244
  18. Capizzi et al. Lidocaine treatment in refractory status epilepticus resulting from FIRES: a case report and follow‑up. 2015. PMID 25343330
  19. Moriyama et al. Protein‑losing enteropathy as a rare complication of the ketogenic diet. 2015. PMID 25724370
  20. Kumari et al. "The great imitator": neurosyphilis and new‑onset refractory status epilepticus (NORSE) syndrome. 2015. PMID 25859427
  21. Amer et al. Refractory status epilepticus from NMDA receptor encephalitis successfully treated with an adjunctive ketogenic diet. 2015. PMID 26019434
  22. Laswell et al. New‑onset refractory status epilepticus in an adult with an atypical presentation of cat‑scratch disease. 2015. PMID 26044784
  23. Agarwal et al. Hippocampal changes in febrile infection‑related epilepsy syndrome (FIRES). 2015. PMID 26379807
2016–2020Case Reports (2016 – 2020)53 articles
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  1. Ogawa et al. Autopsy findings of a patient with acute encephalitis and refractory, repetitive partial seizures. 2016. PMID 26803054
  2. Khawaja et al. Refractory status epilepticus and glutamic acid decarboxylase antibodies in adults: presentation, treatment and outcomes. 2016. PMID 26878120
  3. Sato et al. Acute encephalitis with refractory, repetitive partial seizures: pathological findings and a new therapeutic approach using tacrolimus. 2016. PMID 26906012
  4. Uchida et al. High-dose phenobarbital with intermittent short-acting barbiturates for acute encephalitis with refractory, repetitive partial seizures. 2016. PMID 27273286
  5. Chou et al. Marked improvement in febrile infection‑related epilepsy syndrome after lidocaine plus MgSO₄ treatment in a 12‑year‑old girl. 2016. PMID 27358770
  6. Dillien et al. New‑onset refractory status epilepticus: more investigations, more questions. 2016. PMID 27462243
  7. Kenney Jung et al. Febrile infection‑related epilepsy syndrome treated with anakinra. 2016. PMID 27770579
  8. Zhang et al. Acute encephalitis with refractory, repetitive partial seizures: a case report and literature review. 2016. PMID 27882170
  9. Miras Veiga et al. Effectiveness of electroconvulsive therapy for refractory status epilepticus in FIRES. 2017. PMID 27434885
  10. Uchida et al. Combination of ketogenic diet and stiripentol for super‑refractory status epilepticus. 2017. PMID 28131220
  11. Yamazoe et al. New‑onset refractory status epilepticus treated with vagus nerve stimulation: a case report. 2017. PMID 28267658
  12. Varrasi et al. Auditory seizures in autoimmune epilepsy: a case with anti‑thyroid antibodies. 2017. PMID 28287071
  13. Kaufman et al. Moyamoya in a patient with FIRES: a first case report. 2017. PMID 28436815
  14. Marashly et al. Successful surgical management of NORSE presenting with gelastic seizures in a 3‑year‑old girl. 2017. PMID 28725554
  15. Shrivastava et al. Plasma exchange as a therapeutic modality in a rare case of cryptogenic NORSE. 2017. PMID 28892919
  16. Hau Man et al. Effect of plasmapheresis on serum levels of clobazam, levetiracetam and topiramate. 2017. PMID 28951835
  17. Nardetto et al. 18F‑FDG PET/MRI in cryptogenic NORSE: a potential marker of disease location and activity. 2017. PMID 28991656
  18. Matar et al. New‑onset refractory status epilepticus due to primary angiitis of the central nervous system. 2017. PMID 29062691
  19. Villamar et al. Rabies encephalitis presenting with new‑onset refractory status epilepticus (NORSE). 2017. PMID 29620067
  20. Alparslan et al. FIRES treated with immunomodulation in an 8‑year‑old boy and review of the literature. 2017. PMID 29624228
  21. Kaplan et al. Limbic and new‑onset refractory tonic status epilepticus in anti‑NMDAR encephalitis. 2017. PMID 30214986
  22. Caputo et al. FIRES with super‑refractory status epilepticus revealing autoimmune encephalitis due to GABAAR antibodies. 2018. PMID 29203057
  23. Savard et al. Jamestown Canyon encephalitis with NORSE and electrographic response to ribavirin. 2018. PMID 29881809
  24. Chan et al. Electroconvulsive therapy for new‑onset super‑refractory status epilepticus. 2018. PMID 29926794
  25. Tan et al. FIRES with multifocal subcortical infarcts: a new imaging phenotype. 2018. PMID 29980149
  26. Kodama et al. Favorable outcome of intensive immunotherapies for new‑onset refractory status epilepticus. 2018. PMID 30083346
  27. Agarwal et al. Unusual cause of fever, vision loss and super‑refractory status epilepticus in association with simian virus 40 (SV40). 2018. PMID 30317196
  28. Kurukumbi et al. Vagus nerve stimulation in super‑refractory NORSE. 2019. PMID 30805233
  29. Ungureanu et al. Bilateral fronto‑insular FLAIR hyperintensities in NORSE. 2019. PMID 31004289
  30. Fatuzzo et al. FIRES in an adult patient: an early neuroradiological finding. 2019. PMID 31093785
  31. Dilena et al. Therapeutic effect of anakinra in relapsing chronic phase of FIRES. 2019. PMID 31168503
  32. Eguchi et al. Reversible splenial lesion in NORSE: case report. 2019. PMID 31254907
  33. Al‑Khateeb et al. Schizophrenia following NORSE secondary to antiphospholipid syndrome. 2019. PMID 31380826
  34. Daida et al. NORSE involving limbic system, spinal cord, and peripheral nerves. 2020. PMID 31511489
  35. Hainsworth et al. Super‑refractory status epilepticus treated with high‑dose perampanel. 2019. PMID 31565443
  36. Cho et al. Encephalitis with anti‑SOX1 antibodies presenting with NORSE. 2019. PMID 31591846
  37. Westbrook et al. FIRES treated successfully with anakinra in a 21‑year‑old woman. 2019. PMID 31682750
  38. Mizutani et al. Reversible splenial lesion in a patient with NORSE. 2019. PMID 31909228
  39. Hurth et al. Microdialysis findings in new‑onset refractory non‑convulsive status epilepticus. 2020. PMID 31556003
  40. Monti et al. Anti‑NMDA receptor encephalitis presenting as NORSE in COVID‑19. 2020. PMID 32688169
  41. Eaton et al. Avoiding anaesthetics after multiple failed drug‑induced comas: an unorthodox NORSE management approach. 2020. PMID 31708492
  42. Houk et al. Abdominal compartment syndrome as a rare complication of pentobarbital treatment for NORSE. 2020. PMID 32223826
  43. Brunker et al. NORSE with underlying autoimmune etiology. 2020. PMID 32435752
  44. Ferlisi et al. Very prolonged cryptogenic NORSE with good neurological recovery. 2020. PMID 32458254
  45. Najjar et al. CNS complications associated with SARS‑CoV‑2 infection: integrative concepts and case reports. 2020. PMID 32758257
  46. Goyal et al. Atypical case of FIRES following acute encephalitis: impact of physiotherapy on regaining locomotor abilities. 2020. PMID 32774660
  47. Kern‑Smith et al. The cat's out of the bag: NORSE due to Bartonella henselae. 2020. PMID 32891052
  48. Seniaray et al. 18F‑FDG PET/CT in initial diagnosis and treatment response evaluation of NORSE. 2020. PMID 33082685
  49. Vallecoccia et al. Use of unconventional therapies in super‑refractory status epilepticus: case report and literature review. 2020. PMID 33233961
  50. Kwan et al. Status epilepticus from GABA(B)R antibody positive encephalitis due to mixed small cell and adenocarcinoma of prostate. 2020. PMID 33257388
  51. Jaafar et al. Super‑refractory status epilepticus secondary to anti‑GAD antibody encephalitis successfully treated with immunotherapy. 2020. PMID 33305253
  52. Theroux et al. Improved seizure burden and cognitive performance in a child treated with RNS following FIRES. 2020. PMID 33331273
  53. Stredny et al. Interleukin‑6 blockade with tocilizumab in anakinra‑refractory FIRES. 2020. PMID 33403221
2021–2023Case Reports (2021 – 2023)56 articles
+
  1. Dono et al. New‑onset refractory status epilepticus (NORSE) in post‑SARS‑CoV‑2 autoimmune encephalitis: a case report. 2021. PMID 33145624
  2. Yang et al. Successful treatment of pediatric FIRES with anakinra. 2021. PMID 33227629
  3. Baba et al. Possible role of high‑dose barbiturates and early parenteral ketogenic diet to reduce chronic epilepsy development in FIRES: a case report. 2021. PMID 33231274
  4. Jang et al. Tofacitinib treatment for refractory autoimmune encephalitis. 2021. PMID 33656171
  5. Katz et al. Pearls and pitfalls of introducing ketogenic diet in adult status epilepticus: a guide for intensivists. 2021. PMID 33671485
  6. Meenakshi‑Sundaram et al. Super‑refractory status in FIRES due to hemophagocytic lymphocytic histiocytosis. 2021. PMID 33681644
  7. Donnelly et al. Resolution of cryptogenic new onset refractory status epilepticus with tocilizumab. 2021. PMID 33748736
  8. Cossu et al. Remote teamwork management of NORSE during the COVID‑19 lockdown. 2021. PMID 33842087
  9. Wu et al. Cryptogenic NORSE following blood transfusion in severe anemia. 2021. PMID 33881399
  10. Choi et al. Super‑refractory status epilepticus in autoimmune encephalitis treated with interleukin‑1 receptor antagonist (anakinra). 2021. PMID 34057410
  11. Aladdin et al. New‑onset refractory status epilepticus following the ChAdOx1 nCoV‑19 vaccine. 2021. PMID 34153802
  12. Appleberry et al. Child with febrile status epilepticus and post‑COVID multisystem inflammatory syndrome. 2021. PMID 34285930
  13. Rachfalska et al. Dramatic course of pediatric cryptogenic FIRES with unusual chronic phase presentation and literature review. 2021. PMID 34439649
  14. L'Erario et al. Sevoflurane as bridge therapy for plasma exchange and anakinra in FIRES. 2021. PMID 34596364
  15. Li et al. Ketogenic diet therapy in the acute phase of FIRES: a case report. 2021. PMID 34733679
  16. Nath et al. Role for electroconvulsive therapy in management of NORSE in a young child. 2021. PMID 34747817
  17. Luo et al. Vagus nerve stimulation for super‑refractory status epilepticus in FIRES: pediatric case report and literature review. 2021. PMID 34762158
  18. Shah et al. NORSE as a manifestation of tuberculosis of the CNS. 2021. PMID 34979693
  19. Kumar et al. Anti‑N‑methyl‑D‑aspartate receptor encephalitis presenting as NORSE responding to rituximab in an adolescent girl. 2021. PMID 36531781
  20. Hect et al. Deep brain stimulation of the centromedian thalamic nucleus for treatment of FIRES. 2022. PMID 34862854
  21. Palacios‑Mendoza et al. Response to anakinra in new‑onset refractory status epilepticus: a clinical case. 2022. PMID 34875544
  22. Al‑Chalabi et al. NORSE secondary to HIV CNS viral escape syndrome: case report. 2022. PMID 34894438
  23. Aydemir et al. High‑dose cannabidiol (CBD) in treatment of NORSE. 2022. PMID 34896815
  24. Wadayama et al. Cryptogenic NORSE responded to anti‑interleukin‑6 treatment. 2021. PMID 34973472
  25. Obara et al. Ketogenic diet for a young adult patient with chronic‑phase FIRES. 2022. PMID 35165646
  26. Juneja et al. Evidence‑based approach to diagnosis and prognosis in a young woman with new‑onset super‑refractory status epilepticus. 2022. PMID 35221970
  27. Nawfal et al. New‑onset refractory status epilepticus as early manifestation of multisystem inflammatory syndrome in adults after COVID‑19. 2022. PMID 35306658
  28. Perulli et al. Fighting autoinflammation in FIRES: role of interleukins and early immunomodulation. 2022. PMID 35356746
  29. Orlandi et al. An ultra‑long new‑onset refractory status epilepticus: winning the battle but losing the war. 2022. PMID 35445189
  30. Lala et al. Multifocal multilobar focal cortical dysplasia type IIa in FIRES. 2022. PMID 35483186
  31. Acampora et al. A case of FIRES in a young adult: still a diagnostic and therapeutic challenge. 2022. PMID 35507191
  32. Kurimoto et al. Anti‑inflammatory and immune‑mediated therapy for FIRES with rapid recurrence. 2022. PMID 35685830
  33. Sivathanu et al. Favorable response to classic ketogenic diet in anti‑GAD65 antibody‑mediated super‑refractory status epilepticus. 2022. PMID 35789965
  34. Coban et al. Combined immunoglobulin and plasmapheresis treatment for FIRES. 2022. PMID 35879215
  35. Varughese et al. Initial presentation of PCDH19‑related epilepsy with NORSE treated with anakinra. 2022. PMID 35914944
  36. Basso et al. Connections between FIRES and autoimmune encephalitis: new anti‑neuronal antibodies in a child. 2022. PMID 36003492
  37. Werner et al. NORSE due to autoimmune encephalitis after SARS‑CoV‑2 vaccination: first case report. 2022. PMID 36051224
  38. Chen et al. Role of responsive neurostimulation and immunotherapy in refractory epilepsy due to autoimmune encephalitis. 2022. PMID 36408512
  39. Won et al. The increased interleukin‑6 levels can be an early diagnostic marker for new‑onset refractory status epilepticus. 2022. PMID 36685741
  40. Astner‑Rohracher et al. NORSE in a patient with FASTKD2‑related mitochondrial disease. 2023. PMID 36712458
  41. Kam et al. Inflammatory epilepsy (FIRES) and hemophagocytic lymphohistiocytosis (HLH): an adult case. 2023. PMID 36720516
  42. Bonardi et al. Myoclonic super‑refractory status epilepticus in a teenager with FIRES treated with VNS and cannabidiol. 2023. PMID 36725381
  43. Goh et al. Bridging the gap: tailoring an approach to treatment in FIRES. 2023. PMID 36797068
  44. Rodriguez‑Villar et al. New‑onset refractory status epilepticus responsive to VNS. 2023. PMID 37087686
  45. Indelicato et al. NORSE due to a novel MT‑TF variant: time for acute genetic testing before treatment. 2023. PMID 37090940
  46. Mehta et al. Intrathecal dexamethasone in FIRES: a case report. 2023. PMID 37197372
  47. Guo et al. Claustrum sign in FIRES. 2023. PMID 37273001
  48. Ernst et al. Responsive neurostimulation as treatment for super‑refractory focal status epilepticus: systematic review and case series. 2023. PMID 37329518
  49. Di Dier et al. Claustrum sign in FIRES. 2023. PMID 37396965
  50. Alemdar et al. Anti‑NMDA receptor encephalitis presenting with NORSE. 2023. PMID 37435283
  51. Osbourn et al. NORSE in a traveler with Japanese encephalitis. 2023. PMID 37659268
  52. Sugata et al. Lidocaine as a potential therapeutic option for super‑refractory status epilepticus. 2023. PMID 37692459
  53. Bellingham et al. Multimodal management of FIRES in a 17‑year‑old male. 2023. PMID 37791156
  54. Yang et al. Refractory status epilepticus associated with a pathogenic variant in TNFRSF13B. 2023. PMID 38054159
  55. Cupane et al. Beneficial outcome of subsequent treatment with anakinra during chronic phase of FIRES. 2023. PMID 38132976
  56. Watanabe et al. Reversible Brain Atrophy in Cryptogenic New-onset Refractory Status Epilepticus. 2023. PMID 36351577
2024–2026Case Reports (2024 – 2026)50 articles
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  1. Mehboob et al. Refractory status epilepticus arrested by vagus nerve stimulation. 2024. PMID 37875347
  2. Ruttkowski et al. Febrile infection‑related epilepsy syndrome in a 14‑year‑old girl with multiple organ failure and lethal outcome. 2024. PMID 38505775
  3. Nakamura et al. Treatment of cryptogenic new‑onset refractory status epilepticus (C‑NORSE) with tocilizumab. 2024. PMID 38658340
  4. Sun et al. Electroconvulsive therapy in new‑onset refractory status epilepticus (NORSE) in a pediatric patient. 2024. PMID 38763413
  5. Ohno et al. Steroid‑responsive involuntary movements as a remote symptom of FIRES. 2024. PMID 38887352
  6. Kumar et al. Serial MRI changes in FIRES: a clinical and radiologic exploration. 2024. PMID 38912543
  7. Liverant et al. His brain is on FIRES. 2024. PMID 39033467
  8. Yoneno et al. Efficacy of ketogenic diet in chronic phase FIRES. 2024. PMID 39119419
  9. Yarimi et al. IT‑DEX and B cell depletion in a child with anti‑GAD65 autoimmune encephalitis presenting as NORSE. 2024. PMID 39128431
  10. Harrar et al. Successful management of FIRES using cytokine‑directed therapy. 2024. PMID 39196283
  11. Vrushabhendra et al. A case of FIRES with favorable response to tocilizumab. 2024. PMID 39257172
  12. Shide‑Moriguchi et al. Myelin oligodendrocyte glycoprotein antibody‑associated cerebral cortical encephalitis with super‑refractory status epilepticus. 2024. PMID 39261182
  13. Levee et al. POLG epilepsy presenting as new‑onset refractory status epilepticus (NORSE) in pregnancy. 2025. PMID 39209381
  14. Takahasi et al. Cryptogenic NORSE with bilateral basal ganglia T1‑weighted hyperintensity: autopsy report. 2025. PMID 39343569
  15. Wan et al. Favorable outcomes and FDG‑PET changes after tocilizumab treatment for FIRES in a child. 2025. PMID 39689594
  16. Vaingankar et al. Myelin oligodendrocyte glycoprotein antibody‑associated disease (MOGAD) as a cause of NORSE. 2025. PMID 39799767
  17. Li et al. NORSE secondary to anti‑GAD65 antibody‑positive encephalitis treated with rapid VNS titration protocol. 2025. PMID 39801479
  18. Sudarsanan et al. Early intrathecal dexamethasone associated with faster recovery and good outcome in adolescent FIRES. 2025. PMID 39900388
  19. Ochiai et al. Potential role of perampanel in reducing barbiturate dependency in FIRES: a case report. 2025. PMID 39958143
  20. Horiuchi et al. Status Epilepticus Caused by Tick-borne Encephalitis. 2025. PMID 40128988
  21. Konomatsu et al. Adult‑onset epilepsy with startle‑induced seizure after FIRES. 2025. PMID 40232152
  22. Sabaka et al. Autoimmune encephalitis associated with anti‑SOX1 autoantibodies in COVID‑19. 2025. PMID 40330578
  23. Heinrich et al. Adult‑onset FIRES treated with short‑term anakinra. 2025. PMID 40568054
  24. Kola et al. Rescue use of electroconvulsive therapy in NORSE during pregnancy. 2025. PMID 40774775
  25. Karadjole et al. New‑onset refractory status epilepticus diagnosed in the second trimester. 2025. PMID 41113454
  26. Ding et al. Hemorrhagic cystitis induced by intravenous esketamine in treatment of NORSE in children: a case report. 2025. PMID 41113565
  27. Li et al. Bartonella henselae‑related NORSE/FIRES complicated by stimulus‑induced ictal discharges. 2025. PMID 41124380
  28. Okochi et al. Early recovery from cryptogenic NORSE following repeated C‑NORSE score assessment and cyclophosphamide treatment. 2025. PMID 41125373
  29. Kambe et al. Intensive intracranial pressure and temperature monitoring improving outcome in severe cryptogenic NORSE. 2025. PMID 41181192
  30. Lan et al. Successful treatment of cryptogenic NORSE resistant to immunosuppression with IV ganaxolone and ECT. 2025. PMID 41210562
  31. Sasaki et al. Deep brain stimulation of the anterior thalamic nucleus for seizures after NORSE. 2025. PMID 41245234
  32. Caporale et al. Successful multimodal management of FIRES in a pediatric patient using anakinra, ketogenic diet and induced hypothermia. 2025. PMID 41293210
  33. Takatsu et al. Emergence of atypical lymphocytes before cryptogenic NORSE. 2025. PMID 41306648
  34. Tang et al. Refractory status epilepticus treated with bilateral pulvinar deep brain stimulation. 2025. PMID 41345856
  35. Abdullah et al. Claustrum sign: an increasingly recognized imaging feature of NORSE. 2025. PMID 41357437
  36. Foong et al. Breaking the neuroinflammatory loop with intrathecal dexamethasone in FIRES. 2025. PMID 41454105
  37. Yang et al. Lidocaine as a potential anti‑seizure therapy for pediatric FIRES: a promising approach. 2025. PMID 41480377
  38. Park et al. Advancing FIRES treatment: the potential of intrathecal dexamethasone. 2025. DOI 10.1002/cns3.70008
  39. Nico et al. Rapid recovery after intrathecal dexamethasone in FIRES. 2026. PMID 41502299
  40. Tan et al. FIRES in adults: case report and review of factors associated with survival. 2026. PMID 41524901
  41. McLaughlin et al. Use of electroconvulsive therapy in NORSE: case report and systematic review. 2026. PMID 41557907
  42. Chan et al. Secondary hemophagocytic lymphohistiocytosis concurrent with FIRES in a child. 2026. PMID 41637924
  43. Muccioli et al. Extreme cryptogenic NORSE/FIRES: evidence of profound neuroinflammation and neuronal injury. 2026. PMID 41697173
  44. Mirian et al. Claustrum sign in Hashimoto encephalopathy presenting with NORSE. 2026. PMID 42022976
  45. Bildik et al. Neuroimaging spectrum of immune‑mediated CNS disorders in children following SARS‑CoV‑2 infection — case series component. 2026. PMID 42100917
  46. Meng et al. Superfast VNS titration for the treatment of SRSE in the acute phase of FIRES: a pediatric case report and literature review. 2025. PMID 42261675
  47. Zhang et al. Anakinra for tocilizumab-refractory febrile infection-related epilepsy syndrome with normal IL-1β levels: a case report. 2026. PMID 42367793
  48. Ocakdan et al. Super-refractory Status Epilepticus in Febrile Infection-Related Epilepsy Syndrome Triggered by Influenza A: A Pediatric Case Report. 2026. PMID 42404327
  49. Alamri et al. Febrile infection-related epilepsy syndrome (FIRES): Long-term challenges of phenobarbital management and drug-resistant epilepsy. 2026. PMID 42494178
  50. Chou et al. Case Report: Anti-GABA-B receptor encephalitis with refractory status epilepticus and favorable short-term recovery. 2026. PMID 42614298

Reviews, Pathophysiology, Definitions & Guidelines

Reviews, pathophysiology studies, guidelines, definitions and registry descriptions — 2006 to 2026

2006–2026Others (2006 – 2026)110 articles
+
  1. Sakuma et al. Acute encephalitis with refractory, repetitive partial seizures. 2009. PMID 19327924
  2. Kobayashi et al. Genetic seizure susceptibility underlying acute encephalopathies in childhood. 2010. PMID 20675100
  3. Nabbout et al. Acute encephalopathy with inflammation-mediated status epilepticus. 2011. PMID 21163447
  4. Suleiman et al. VGKC antibodies in pediatric encephalitis presenting with status epilepticus. 2011. PMID 21464429
  5. Carranza Rojo et al. Febrile infection-related epilepsy syndrome is not caused by SCN1A mutations. 2012. PMID 22386634
  6. Kobayashi et al. Acute encephalopathy with a novel point mutation in the SCN2A gene. 2012. PMID 22591750
  7. Van Baalen et al. Febrile Infection-Related Epilepsy Syndrome without Detectable Autoantibodies and Response to Immunotherapy. 2012. PMID 22911482
  8. Appenzeller et al. Febrile infection-related epilepsy syndrome (FIRES) is not caused by SCN1A, POLG, PCDH19 mutations or rare copy number variations. 2012. PMID 23066759
  9. Nabbout et al. FIRES and IHHE: Delineation of the syndromes. 2013. PMID 24001074
  10. Pardo et al. Mechanisms of Epileptogenesis in Pediatric Epileptic Syndromes: Rasmussen Encephalitis, Infantile Spasms, and Febrile Infection-related Epilepsy Syndrome (FIRES). 2014. PMID 24639375
  11. Sakuma et al. Intrathecal overproduction of proinflammatory cytokines and chemokines in febrile infection-related refractory status epilepticus. 2015. PMID 25398790
  12. Saitoh et al. Cytokine-related and sodium channel polymorphism as candidate predisposing factors for childhood encephalopathy FIRES/AERRPS. 2016. PMID 27538648
  13. Van Baalen et al. Febrile Infection–Related Epilepsy Syndrome: Clinical Review and Hypotheses of Epileptogenesis. 2017. PMID 27919115
  14. Hirsch et al. Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. 2018. PMID 29399791
  15. Gaspard et al. New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): State of the art and perspectives. 2018. PMID 29476535
  16. Hon et al. Febrile Infection-Related Epilepsy Syndrome (FIRES): An Overview of Treatment and Recent Patents. 2018. PMID 29745347
  17. Sculier et al. New onset refractory status epilepticus (NORSE). 2019. PMID 30482654
  18. Clarkson et al. Functional Deficiency in Endogenous Interleukin-1 Receptor Antagonist in Patients with Febrile Infection-Related Epilepsy Syndrome. 2019. PMID 30779222
  19. Culleton et al. The spectrum of neuroimaging findings in febrile infection-related epilepsy syndrome (FIRES): A literature review. 2019. PMID 30854647
  20. Gofton et al. New onset refractory status epilepticus research: What is on the horizon? 2019. PMID 30894443
  21. Gofton et al. Communication Challenges: A Spotlight on New-Onset Refractory Status Epilepticus. 2019. PMID 30935709
  22. Kothur et al. 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. 2019. PMID 31283843
  23. Serino et al. Febrile infection-related epilepsy syndrome (FIRES): prevalence, impact and management strategies. 2019. PMID 31371963
  24. Payne et al. Extinguishing Febrile Infection-Related Epilepsy Syndrome: Pipe Dream or Reality? 2020. PMID 32185792
  25. Sakuma et al. Neurocritical care and target immunotherapy for febrile infection-related epilepsy syndrome. 2020. PMID 32330681
  26. Specchio et al. New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome. 2020. PMID 32372459
  27. Kessi et al. Efficacy of different treatment modalities for acute and chronic phases of the febrile infection-related epilepsy syndrome: A systematic review. 2020. PMID 32417686
  28. Hsieh et al. Diminished toll-like receptor response in febrile infection-related epilepsy syndrome (FIRES). 2020. PMID 32651134
  29. Helbig et al. Whole-exome and HLA sequencing in Febrile infection-related epilepsy syndrome. 2020. PMID 32666661
  30. Bhatia et al. New Onset Refractory Status Epilepticus. 2023. PMID 33620842
  31. Mantoan et al. New-onset refractory status epilepticus (NORSE). 2021. PMID 33674412
  32. Koh et al. Proposal to optimize evaluation and treatment of Febrile infection-related epilepsy syndrome (FIRES): A Report from FIRES workshop. 2021. PMID 33681649
  33. Kazazian et al. How to Help Your Patients Enroll in the NORSE and FIRES Family Registry, and Other Rare Epilepsy Registries. 2021. PMID 33769129
  34. Farias Moeller et al. Parental perception of FIRES outcomes, emotional states, and social media usage. 2021. PMID 34098587
  35. Lin et al. Hypothesis: Febrile infection-related epilepsy syndrome is a microglial NLRP3 inflammasome/IL-1 axis-driven autoinflammatory syndrome. 2021. PMID 34141434
  36. Yamanaka et al. Towards a Treatment for Neuroinflammation in Epilepsy: Interleukin-1 Receptor Antagonist, Anakinra, as a Potential Treatment in Intractable Epilepsy. 2021. PMID 34208064
  37. Lattanzi et al. Unraveling the enigma of new-onset refractory status epilepticus: a systematic review of aetiologies. 2022. PMID 34661330
  38. Périn et al. New onset refractory status epilepticus: State of the art. 2022. PMID 35031143
  39. Specchio et al. International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood. 2022. PMID 35503717
  40. Wickstrom et al. International consensus recommendations for management of NORSE including FIRES: Summary and Clinical Tools. 2022. PMID 35951466
  41. Wickstrom et al. International consensus recommendations for management of NORSE including FIRES: Statements and Supporting Evidence. 2022. PMID 35997591
  42. Arif et al. New-Onset Refractory Status Epilepticus Secondary to COVID-19 Infection in Adults: A Systematic Review. 2022. PMID 36065386
  43. Kaur et al. Fever, Seizures and Encephalopathy: From Bush Fires to Firestorms. 2022. PMID 36211150
  44. Wang et al. Multi-proteomic Analysis Revealed Distinct Protein Profiles in Cerebrospinal Fluid of Patients Between Anti-NMDAR Encephalitis NORSE and Cryptogenic NORSE. 2023. PMID 36224320
  45. Lin et al. The nuances of immunotherapy for NORSE/FIRES. 2022. PMID 36266948
  46. Wong et al. Surviving status epilepticus: The patient and family perspective. 2023. PMID 36521408
  47. Tharmaraja et al. The etiology and mortality of new-onset refractory status epilepticus (NORSE) in adults: A systematic review and meta-analysis. 2023. PMID 36727541
  48. Taraschenko et al. Seizure burden and neuropsychological outcomes of new-onset refractory status epilepticus: Systematic review. 2023. PMID 36761344
  49. Sculier et al. New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome. 2023. PMID 36762646
  50. Gaspard et al. Is NORSE a meaningful clinical entity? 2023. PMID 36807989
  51. Hanin et al. Cytokines in New-Onset Refractory Status Epilepticus Predict Outcomes. 2023. PMID 36871188
  52. Li et al. Blood–brain barrier damage and new onset refractory status epilepticus: An exploratory study using dynamic contrast-enhanced magnetic resonance imaging. 2023. PMID 36892496
  53. Howe et al. Drug-resistant seizures associated with hyperinflammatory monocytes in FIRES. 2023. PMID 36924141
  54. Gopaul et al. The seasonality of new-onset refractory status epilepticus (NORSE). 2023. PMID 37013696
  55. Cerovic et al. An in vitro model of drug-resistant seizures for selecting clinically effective antiseizure medications in Febrile Infection-Related Epilepsy Syndrome. 2023. PMID 37034097
  56. Hanin et al. Review and standard operating procedures for collection of biospecimens and analysis of biomarkers in new onset refractory status epilepticus. 2023. PMID 37039049
  57. DeCampo et al. Investigating the genetic contribution in febrile infection-related epilepsy syndrome and refractory status epilepticus. 2023. PMID 37077567
  58. Hanin et al. Neuropathology of New-Onset Refractory Status Epilepticus (NORSE). 2023. PMID 37079033
  59. Vinette et al. Early identification of NORSE and transfer to care setting with appropriate supports: A proposed algorithm. 2023. PMID 37114231
  60. Mantoan et al. Perspective: Vagal nerve stimulation in the treatment of new-onset refractory status epilepticus. 2023. PMID 37153661
  61. Sheikh et al. A practical approach to in-hospital management of new-onset refractory status epilepticus/febrile infection related epilepsy syndrome. 2023. PMID 37251223
  62. Dale et al. CSF neopterin and quinolinic acid are biomarkers of neuroinflammation and neurotoxicity in FIRES and other infection-triggered encephalopathy syndromes. 2023. PMID 37340737
  63. Stavropoulos et al. Neuromodulation in new-onset refractory status epilepticus. 2023. PMID 37388544
  64. Shin et al. Understanding new-onset refractory status epilepticus from an immunological point of view. 2021. PMID 37469848
  65. Van Baalen et al. Febrile infection-related epilepsy syndrome in childhood: A clinical review and practical approach. 2023. PMID 37703593
  66. Thomschewski et al. Editorial: Advances in diagnosing and treating new-onset refractory status epilepticus (NORSE). 2023. PMID 37712084
  67. Kang et al. Interleukin-1 in Febrile Infection-Related Epilepsy Syndrome. 2023. PMID 37712084
  68. Carson et al. New-Onset Refractory Status Epilepticus and Febrile Infection-Related Epilepsy Syndrome. 2023. PMID 37951651
  69. Hanin et al. New onset refractory status epilepticus: Summary of the NORSE Institute roundtable during the 2023 American Epilepsy Society meeting. 2024. PMID 38421053
  70. Hanin et al. Second-line immunotherapy in new onset refractory status epilepticus. 2024. PMID 38430119
  71. Bolle et al. NORSE seasonality may vary geographically in adults. 2024. PMID 38635008
  72. Hanin et al. Single-Cell Transcriptomic Analyses of Brain Parenchyma in Patients With NORSE. 2024. PMID 38810181
  73. Cabezudo-Garcia et al. Functional outcomes of patients with NORSE and FIRES treated with immunotherapy: A systematic review. 2024. PMID 36155099
  74. Champsas et al. NORSE/FIRES: how can we advance our understanding of this devastating condition? 2024. PMID 39175762
  75. Kazazian et al. Communication trends over time in new-onset refractory status epilepticus (NORSE): Interim analysis from the NORSE/FIRES Family Registry. 2024. PMID 39241640
  76. Jang et al. Polygenic Landscape of Cryptogenic New-Onset Refractory Status Epilepticus: A Comprehensive Whole-Genome Sequencing Study. 2024. PMID 39440581
  77. Stavropoulos et al. New-onset refractory status epilepticus after SARS-CoV-2 infection: a review of literature. 2024. PMID 39812100
  78. Eschbach et al. Navigating life after NORSE and FIRES: Insights from caregiver and patient interviews. 2025. PMID 39740258
  79. Kazazian et al. Age-associated differences in FIRES: Characterizing prodromal presentation and long-term outcomes via the web-based NORSE/FIRES Family Registry. 2025. PMID 39804054
  80. Espino et al. New onset refractory status epilepticus: Long-term outcomes beyond seizures. 2025. PMID 39825688
  81. Goh et al. CSF Tau Is a Biomarker of Hippocampal Injury in Cryptogenic New-Onset Refractory Status Epilepticus. 2025. PMID 40164513
  82. Wang et al. Potential inflammatory mechanisms of the ketogenic diet against febrile infection-related epilepsy syndrome. 2025. PMID 40217546
  83. Guillemaud et al. Identification of Distinct Biological Groups of Patients With Cryptogenic NORSE via Inflammatory Profiling. 2025. PMID 40334176
  84. Steriade et al. Patients with status epilepticus and new-onset refractory status epilepticus display drastically altered fecal microbiomes compared to chronic epilepsy patients. 2025. PMID 40387216
  85. Hanin et al. Immune dysregulation in new-onset refractory status epilepticus (NORSE): current insights and therapeutic perspectives. 2025. PMID 40712353
  86. Shah et al. Update in new-onset refractory status epilepticus and febrile infection-related epilepsy syndrome. 2025. PMID 40831349
  87. Guillemaud et al. Standard complete blood count to predict long-term outcomes in febrile infection-related epilepsy syndrome (FIRES): A multicenter study. 2025. PMID 40844418
  88. Fisher et al. De novo pathogenic CSF1R variant implicates microglial dysfunction in pathogenesis of febrile infection-related epilepsy syndrome. 2025. PMID 40956030
  89. Al Wahaibi et al. The spectrum of cognitive outcomes following admission to hospital for refractory status epilepticus (RSE): A scoping review. 2025. PMID 40972237
  90. Suri-Baez et al. Beyond Anakinra and Tocilizumab: Additional Adjunctive Therapies in Pediatric NORSE and FIRES - A Narrative Review. 2026. PMID 41265024
  91. Hanin et al. Exploring pathways leading to drug-resistant epilepsy for patients with cryptogenic new onset refractory status epilepticus. 2026. PMID 41697646
  92. Nasuno et al. An increase in cerebrospinal fluid macrophage migration inhibitory factor levels in pediatric patients with acute neuroinflammatory diseases. 2026. PMID 41707435
  93. Meletti et al. Acute Brain Injury in New-Onset Refractory Status Epilepticus and Etiology-Defined Status Epilepticus. 2026. PMID 42043830
  94. Konomatsu et al. Vagus nerve stimulation markedly reduced startle-induced seizures after febrile infection-related epilepsy syndrome. 2025. PMID 42216995
  95. Eschbach et al. New-onset refractory status epilepticus/febrile infection-related epilepsy syndrome centers of excellence: a framework for coordinated care, research, and improved outcomes. 2025. PMID 42257690
  96. Farias-Moeller & Lai. Biomarkers of new onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES). 2026. DOI 10.1016/j.spen.2026.101298
  97. Hanin et al. Insights into new-onset refractory status epilepticus (NORSE) from biorepository-based studies. 2026. DOI 10.1016/j.spen.2026.101296
  98. Eschbach & Gofton. The post-acute phase of new-onset refractory status epilepticus (NORSE) / febrile infection-related epilepsy syndrome (FIRES). 2026. DOI 10.1016/j.spen.2026.101317
  99. Fisher & Muscal. Immunotherapies for NORSE and FIRES. 2026. DOI 10.1016/j.spen.2026.101295
  100. Wickström et al. Recognizing NORSE and FIRES — clinical course, diagnosis and evaluation. 2026. DOI 10.1016/j.spen.2026.101294
  101. Thomas et al. The creation of an international NORSE/FIRES virtual consultation forum. 2026. DOI 10.1016/j.spen.2026.101297
  102. Wong et al. Physician–Family Communication in New-Onset Refractory Status Epilepticus (NORSE): Communicating when there are few answers. 2026. DOI 10.1016/j.spen.2026.101319
  103. Phong et al. Time-dependent changes in cerebrospinal fluid cytokines in NORSE and FIRES: A systematic review. 2026. DOI 10.1016/j.seizure.2026.07.001
  104. Gettings et al. Symptomatic Management of NORSE/FIRES: Acute and Chronic Anti-seizure Treatment. 2026. DOI 10.1016/j.spen.2026.101320
  105. Mantoan Ritter et al. Neuromodulation in NORSE/FIRES. 2026. DOI 10.1016/j.spen.2026.101322
  106. Kuo et al. Vagus nerve stimulation for refractory status epilepticus: A systematic review. 2026. PMID 42531696
  107. Wang et al. Total bilirubin-to-albumin ratio and systemic immune inflammation index as prognostic indicators in children with febrile infection-related epilepsy syndrome. 2026. PMID 42551214
  108. Burani et al. Claustrum Involvement in New Onset Refractory Status Epilepticus: A Systematic Review. 2026. PMID 42552664
  109. Phong et al. Adaptive immune dysregulation drives new-onset refractory status epilepticus. 2026. PMID 42597690
  110. Eyre et al. Infection-triggered encephalopathy syndromes: a meta-analysis of clinical characteristics and outcomes in 1946 cases. 2026. PMID 42699010

Full chronological list (2001–2026) — for reference and database integration.

© 2026NORSE-ICM Project – Paris Brain Institute (ICM)