Long-term outcomes of adult cryptogenic febrile infection-related epilepsy syndrome (FIRES).
Long-term outcomes of adult cryptogenic febrile infection-related epilepsy syndrome (FIRES).
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DOI：
10.3389/fneur.2022.1081388
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发表时间：
2022
影响因子：
3.4
通讯作者：
Jiang, Wen
中科院分区：
文献类型：
--
作者：
Shi, Xiaojing;Wang, Yuanyuan;Wang, Xuan;Kang, Xiaogang;Yang, Fang;Yuan, Fang;Jiang, Wen
关键词：
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。我们的目的是研究成人隐源性火灾的长期结果。这是一项基于中国三级医院神经重症监护室前瞻性数据库的回顾性研究。包括 2007 年 7 月至 2021 年 12 月期间连续发生隐源性 FIRES 的成年患者。长期结果包括功能独立性、耐药性癫痫 (DRE)、远程复发性癫痫持续状态 (SE)、抗癫痫药物 (ASM) 以及大脑磁共振成像 (MRI) 的变化。从 270 名 SE 患者中总共鉴定出 11 名患有隐源性 FIRES 的成人患者。 4 名患者 (36%) 在医院死亡，其中 3 名患者退出治疗，1 名患者在出院 12 个月后死亡。经过12至112个月的随访，6名（55%）患者仍存活，并且全部实现功能独立[改良Rankin量表（mRS）0-3]。 45% (5/11) 的患者出现 DRE，18% (2/11) 的患者出现远端复发性 SE，55% (6/11) 的患者在最后一次随访时接受 ASM 联合治疗。大多数最初 MRI 正常或异常的患者在随访时海马体出现异常，而急性期发现的大多数其他 MRI 异常随着时间的推移而消失。成人隐源性火灾的后果是令人畏惧的。超过三分之一的患者在医院死亡。隐源性 FIRES 的幸存者可能会恢复功能独立，但他们通常会出现 DRE 并长期接受 ASM 的多种治疗。
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.
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