Service de Neurologie et Centre de Référence pour le Traitement de l'Epilepsie Réfractaire, Université Libre de Bruxelles - Hôpital Erasme, Bruxelles, Belgique.
Department of Neurology, Comprehensive Epilepsy Center, Yale University School of Medicine, New Haven, CT, USA.
Epilepsia. 2018 Apr;59(4):745-752. doi: 10.1111/epi.14022. Epub 2018 Feb 24.
We report the proceedings of the First International new-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) Symposium. To promote awareness of this condition and foster research efforts, we conveyed the First International new-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) Symposium. The conference was supported by The NORSE Institute (http://www.norseinstitute.org). This article summarizes the discussions that were held during the Symposium and presents our strategy to unravel the cause of these disorders and to improve patient care. The standardized definitions for these disorders that have been developed, are required to improve communication and facilitate the development of multicenter registries and biobanks. A distinction between childhood- and adult-onset forms of the syndrome is not supported by strong scientific evidence and it is argued that both should be studied together. Although the pathophysiology remains elusive, nascent evidence suggests a role for a postinfectious cytokine-mediated mechanism, which should be further investigated. It also appears important to develop tools for their early recognition and prompt treatment. Recent evidence suggests that specific electroencephalography (EEG) features might be helpful. The optimal treatment options remain to be determined; immune therapies are usually disappointing, but the ketogenic diet has proved effective in uncontrolled trials. NORSE and FIRES represent a very delicate clinical situation with specific communication issues between physicians and with patients and families. Standardized consensus definitions and a multidisciplinary multicenter strategy will help research efforts and improve clinical care for patients with NORSE and FIRES.
我们报告了第一届新发病例难治性癫痫持续状态（NORSE）和发热性感染相关癫痫综合征（FIRES）研讨会的会议记录。为了提高对这种疾病的认识并促进研究工作，我们传达了第一届新发病例难治性癫痫持续状态（NORSE）和发热性感染相关癫痫综合征（FIRES）研讨会的会议记录。该会议得到了 NORSE 研究所（http://www.norseinstitute.org）的支持。本文总结了研讨会期间的讨论，并提出了我们解开这些疾病病因并改善患者护理的策略。为了改善沟通并促进多中心登记和生物库的发展，需要制定这些疾病的标准化定义。没有强有力的科学证据支持将儿童期和成年期发病的综合征区分开来，因此应该一起进行研究。尽管病理生理学仍然难以捉摸，但新出现的证据表明，一种感染后细胞因子介导的机制可能起作用，应该进一步研究。似乎也很重要的是，开发用于早期识别和及时治疗的工具。最近的证据表明，特定的脑电图（EEG）特征可能会有所帮助。最佳治疗选择仍有待确定；免疫疗法通常令人失望，但生酮饮食已被证明在对照试验中有效。NORSE 和 FIRES 代表了一种非常微妙的临床情况，医生与患者和家属之间存在特定的沟通问题。标准化的共识定义和多学科多中心策略将有助于研究工作并改善 NORSE 和 FIRES 患者的临床护理。
