Service de Neurologie Pédiatrique, Hôpital Universitaire de Bruxelles - Hôpital Erasme.
Service de Neurologie, Hôpital Universitaire de Bruxelles - Hôpital Erasme, Bruxelles, Belgique.
Curr Opin Neurol. 2023 Apr 1;36(2):110-116. doi: 10.1097/WCO.0000000000001137. Epub 2023 Feb 10.
The concept and understanding of new-onset refractory status epilepticus (NORSE), and its subtype with prior fever known as febrile infection-related epilepsy syndrome (FIRES) have evolved in the recent past. This review aims to summarize the recent developments in the pathophysiology, diagnosis and management of these challenging conditions.
NORSE and FIRES can have many different causes. Although the list of possible causes is still growing, they mostly fall in the categories of autoimmune encephalitis and genetic disorders. However, despite extensive investigations, most cases of NORSE and FIRES remain cryptogenic. Recent studies have pointed towards the key role of autoinflammation as a unifying pathophysiological mechanism in these cases. These findings also support the use of immunomodulatory treatment in this setting. Consensus recommendations on the management of NORSE and FIRES have recently been published.
NORSE and FIRES remain challenging conditions to diagnose and treat. Recent findings from clinical and basic research and new recommendations, reviewed in this article, contribute to an emerging framework for management and future research.
新起难治性癫痫持续状态（NORSE）及其先前有发热病史的亚型——热性感染相关癫痫综合征（FIRES）的概念和理解在最近发生了演变。本篇综述旨在总结这些具有挑战性病症的病理生理学、诊断和治疗方面的最新进展。
NORSE 和 FIRES 可能有许多不同的病因。尽管可能的病因列表仍在不断增加，但它们大多属于自身免疫性脑炎和遗传疾病类别。然而，尽管进行了广泛的调查，NORSE 和 FIRES 的大多数病例仍然是病因不明的。最近的研究指出，自身炎症在这些情况下是一种统一的病理生理机制，支持在此类情况下使用免疫调节治疗。最近发表了关于 NORSE 和 FIRES 管理的共识建议。
NORSE 和 FIRES 仍然是诊断和治疗具有挑战性的病症。本文回顾的临床和基础研究的最新发现以及新的建议，为管理和未来研究提供了一个新兴的框架。
