热性感染相关性癫痫综合征

Febrile infection-related epilepsy syndrome

摘要

热性感染相关性癫痫综合征（febrile infection-related epilepsy syndrome，FIRES）是一种发生在既往健康的儿童和青少年的炎症相关急性脑病，以热性感染后出现的超级难治性癫痫持续状态为临床特征，多数预后不良。近年来随着对该病认识的加深，治疗理念也有所转变，更提倡在规范抗癫痫发作治疗的基础上，早期及时免疫治疗和综合支持。本文从FIRES的定义、临床特征、辅助检查、发病机制、诊断及评估、治疗、预后和未来研究方向进行了全面阐述，以期提高认识，改善预后。

Abstract

Febrile infection-related epilepsy syndrome is an inflammation-related acute encephalopathy that occurs in previously healthy children and adolescents. It is clinically characterized by super-refractory status epilepticus that occurs after a febrile infection and is often associated with poor prognosis. In recent years，with a deeper understanding of this disease，the treatment approaches have shifted to early and timely immunotherapy and comprehensive support on the basis of standardized anti-epileptic seizure treatment. This review comprehensively elaborates the definition，clinical characteristics，auxiliary examinations，pathogenesis，diagnosis and evaluation，treatment，prognosis，and future research directions of febrile infection-related epilepsy syndrome，aiming to enhance understanding and improve prognosis.

关键词

热性感染相关性癫痫综合征 / 新发生的难治性癫痫持续状态 / 感染诱发性脑病综合征

Key words

febrile infection-related epilepsy syndrome / new-onset refractory status epilepticus / infection-triggered encephalopathy syndrome