G Giussani , E Bianchi , CM Bonardi , S Sartori , S Buratti , E Campostrini , G Cantalupo , AL Ghobert , CA Cesaroni , A Fetta , E Freri , L Giordano , S Olivotto , G Toscano , A Papa , F Brustia , M Vissani , S Micheli , R Dilena
{"title":"anakinra治疗隐源性NORSE/FIRES患者的回顾性多中心研究","authors":"G Giussani , E Bianchi , CM Bonardi , S Sartori , S Buratti , E Campostrini , G Cantalupo , AL Ghobert , CA Cesaroni , A Fetta , E Freri , L Giordano , S Olivotto , G Toscano , A Papa , F Brustia , M Vissani , S Micheli , R Dilena","doi":"10.1016/j.seizure.2025.06.022","DOIUrl":null,"url":null,"abstract":"<div><h3>Purpose</h3><div>Refractory status epilepticus (RSE) is a neurological emergency characterized by persistent seizures despite two appropriate antiseizure medications. It is defined as new-onset RSE (NORSE) when occurring in previously healthy people. NORSE is defined as cryptogenic (cNORSE) when no cause is found. Febrile‐infection related epilepsy syndrome (FIRES) is now defined as the cNORSE variant preceded by fever. In 2016 anakinra was reported effective in an anecdotal case. After this experience, second-line immunotherapy has been increasingly used, being incorporated in cNORSE recommendations in 2022.</div></div><div><h3>Methods</h3><div>We conducted a retrospective study in an Italian case series of 14 patients with cNORSE (including FIRES cases) treated with anakinra in Italy before implementation of the international recommendations, that could serve as historical reference for futures studies based on the shared recommendations.</div></div><div><h3>Results</h3><div>In our population higher duration of intensive care stay period was associated with later time of anakinra use, although no evidence of efficacy on the long-term neurological outcome was demonstrated.</div></div><div><h3>Conclusion</h3><div>There is an urgent need for well-designed head-to-head multicenter prospective clinical trials to clarify the benefits of available treatments and the role of biomarkers to guide choices.</div></div>","PeriodicalId":49552,"journal":{"name":"Seizure-European Journal of Epilepsy","volume":"130 ","pages":"Pages 141-146"},"PeriodicalIF":2.7000,"publicationDate":"2025-06-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Retrospective multicenter study on cryptogenic NORSE/FIRES patients treated with anakinra\",\"authors\":\"G Giussani , E Bianchi , CM Bonardi , S Sartori , S Buratti , E Campostrini , G Cantalupo , AL Ghobert , CA Cesaroni , A Fetta , E Freri , L Giordano , S Olivotto , G Toscano , A Papa , F Brustia , M Vissani , S Micheli , R Dilena\",\"doi\":\"10.1016/j.seizure.2025.06.022\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><h3>Purpose</h3><div>Refractory status epilepticus (RSE) is a neurological emergency characterized by persistent seizures despite two appropriate antiseizure medications. It is defined as new-onset RSE (NORSE) when occurring in previously healthy people. NORSE is defined as cryptogenic (cNORSE) when no cause is found. Febrile‐infection related epilepsy syndrome (FIRES) is now defined as the cNORSE variant preceded by fever. In 2016 anakinra was reported effective in an anecdotal case. After this experience, second-line immunotherapy has been increasingly used, being incorporated in cNORSE recommendations in 2022.</div></div><div><h3>Methods</h3><div>We conducted a retrospective study in an Italian case series of 14 patients with cNORSE (including FIRES cases) treated with anakinra in Italy before implementation of the international recommendations, that could serve as historical reference for futures studies based on the shared recommendations.</div></div><div><h3>Results</h3><div>In our population higher duration of intensive care stay period was associated with later time of anakinra use, although no evidence of efficacy on the long-term neurological outcome was demonstrated.</div></div><div><h3>Conclusion</h3><div>There is an urgent need for well-designed head-to-head multicenter prospective clinical trials to clarify the benefits of available treatments and the role of biomarkers to guide choices.</div></div>\",\"PeriodicalId\":49552,\"journal\":{\"name\":\"Seizure-European Journal of Epilepsy\",\"volume\":\"130 \",\"pages\":\"Pages 141-146\"},\"PeriodicalIF\":2.7000,\"publicationDate\":\"2025-06-24\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Seizure-European Journal of Epilepsy\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S1059131125001712\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q2\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Seizure-European Journal of Epilepsy","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S1059131125001712","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
Retrospective multicenter study on cryptogenic NORSE/FIRES patients treated with anakinra
Purpose
Refractory status epilepticus (RSE) is a neurological emergency characterized by persistent seizures despite two appropriate antiseizure medications. It is defined as new-onset RSE (NORSE) when occurring in previously healthy people. NORSE is defined as cryptogenic (cNORSE) when no cause is found. Febrile‐infection related epilepsy syndrome (FIRES) is now defined as the cNORSE variant preceded by fever. In 2016 anakinra was reported effective in an anecdotal case. After this experience, second-line immunotherapy has been increasingly used, being incorporated in cNORSE recommendations in 2022.
Methods
We conducted a retrospective study in an Italian case series of 14 patients with cNORSE (including FIRES cases) treated with anakinra in Italy before implementation of the international recommendations, that could serve as historical reference for futures studies based on the shared recommendations.
Results
In our population higher duration of intensive care stay period was associated with later time of anakinra use, although no evidence of efficacy on the long-term neurological outcome was demonstrated.
Conclusion
There is an urgent need for well-designed head-to-head multicenter prospective clinical trials to clarify the benefits of available treatments and the role of biomarkers to guide choices.
期刊介绍:
Seizure - European Journal of Epilepsy is an international journal owned by Epilepsy Action (the largest member led epilepsy organisation in the UK). It provides a forum for papers on all topics related to epilepsy and seizure disorders.