Shi Xiaojing, Wang Yuanyuan, Wang Xuan, Kang Xiaogang, Yang Fang, Yuan Fang, Jiang Wen
Department of Neurology, Xijing Hospital, Fourth Military Medical University, Xi'an, China.
Department of Neurology, The Second Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou, China.
Front Neurol. 2023 Jan 4;13:1081388. doi: 10.3389/fneur.2022.1081388. eCollection 2022.
Cryptogenic febrile infection-related epilepsy syndrome (FIRES) is a rare but catastrophic encephalopathic condition. We aimed to investigate the long-term outcome in adult cryptogenic FIRES.
This was a retrospective study based on the prospective database in the neuro-intensive care unit of a tertiary hospital in China. Consecutive adult patients with cryptogenic FIRES between July 2007 to December 2021 were included. Long-term outcomes included function independence, the development of drug-resistant epilepsy (DRE), remote recurrent status epilepticus (SE), anti-seizure medications (ASMs), and changes in the brain Magnetic Resonance Imaging (MRI).
A total of 11 adult patients with cryptogenic FIRES were identified from 270 patients with SE. Four (36%) patients died in the hospital, with three of them withdrawing treatments, and one patient died 12 months after discharge. After the follow-up ranging from 12 to 112 months, 6 (55%) patients were still alive, and all of them achieved functional independence [modified Rankin Scale (mRS) 0-3]. 45% (5/11) patients developed DRE, 18% (2/11) had remote recurrent SE, and 55% (6/11) were on polytherapy with ASMs at the last follow-up. Most of the patients with initial normal or abnormal MRI had abnormalities in the hippocampus at follow-up, and most of the other MRI abnormalities found in the acute stage disappeared over time.
The outcome of adult cryptogenic FIRES is daunting. More than one-third of patients die in the hospital. Survivors of cryptogenic FIRES may regain functional independence, but they usually develop DRE and receive polytherapy of ASMs for a long time.
隐源性发热感染相关癫痫综合征（FIRES）是一种罕见但灾难性的脑病。我们旨在研究成人隐源性FIRES的长期预后。
这是一项基于中国一家三级医院神经重症监护病房前瞻性数据库的回顾性研究。纳入2007年7月至2021年12月期间连续的成年隐源性FIRES患者。长期预后包括功能独立性、耐药性癫痫（DRE）的发生、远期癫痫持续状态（SE）复发、抗癫痫药物（ASM）使用情况以及脑磁共振成像（MRI）的变化。
在270例癫痫持续状态患者中，共识别出11例成年隐源性FIRES患者。4例（36%）患者在医院死亡，其中3例放弃治疗，1例患者出院12个月后死亡。经过12至112个月的随访，6例（55%）患者仍存活，且均实现了功能独立[改良Rankin量表（mRS）0 - 3]。45%（5/11）的患者发生了DRE，18%（2/11）有远期SE复发，55%（6/11）的患者在最后一次随访时接受多种ASM联合治疗。大多数初始MRI正常或异常的患者在随访时海马区出现异常，急性期发现的大多数其他MRI异常随时间消失。
成人隐源性FIRES的预后令人沮丧。超过三分之一的患者在医院死亡。隐源性FIRES的幸存者可能恢复功能独立，但通常会发生DRE并长期接受多种ASM联合治疗。
