anakinra治疗隐源性NORSE/FIRES患者的回顾性多中心研究

IF 2.7 3区 医学 Q2 CLINICAL NEUROLOGY
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
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引用次数: 0

摘要

目的:难治性癫痫持续状态(RSE)是一种以持续癫痫发作为特征的神经系统急症,尽管有两种适当的抗癫痫药物。它被定义为新发RSE (NORSE),当发生在以前健康的人。当没有发现原因时,NORSE被定义为隐源性(cNORSE)。发热感染相关癫痫综合征(FIRES)现在定义为伴有发热的cNORSE变体。2016年,阿那金在一个轶事案例中被报道有效。在这一经验之后,二线免疫疗法的使用越来越多,并于2022年被纳入cNORSE建议。方法在国际推荐标准实施前,我们对意大利14例接受阿那金治疗的cNORSE患者(包括FIRES病例)进行回顾性研究,为今后基于共同推荐标准的研究提供历史参考。结果在我们的人群中,重症监护时间越长,阿那白的使用时间越晚,尽管没有证据表明对长期神经预后有疗效。结论迫切需要精心设计的正面多中心前瞻性临床试验,以明确现有治疗方法的益处和生物标志物的作用,以指导选择。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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cases) treated with anakinra in Italy before implementation of the international recommendations, that could serve as historical reference for futures studies based on the shared recommendations.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.
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来源期刊
Seizure-European Journal of Epilepsy
Seizure-European Journal of Epilepsy 医学-临床神经学
CiteScore
5.60
自引率
6.70%
发文量
231
审稿时长
34 days
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.
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