Department of Neuropediatrics, University Medical Center Schleswig-Holstein, Christian-Albrechts-Universität zu Kiel, Kiel, Germany.
Epilepsia. 2010 Jul;51(7):1323-8. doi: 10.1111/j.1528-1167.2010.02535.x. Epub 2010 Mar 18.
Encephalitis is generally presumed, even when seizures follow banal febrile infection, and pathogen detection in cerebrospinal fluid fails. This retrospective multicenter case series reports on 22 previously healthy children aged 3-15 years (median 6.5 years) with prolonged or recurrent seizures occurring 2-14 days (median 5 days) after fever onset (19 children with respiratory or nonspecific infections). Cerebrospinal fluid studies revealed 2-42 cells/microl (median 5 cells/microl) and no pathogens. Electroencephalography showed diffuse slowing or multifocal discharges. Neuroimaging demonstrated normal findings in 10 children. Brain biopsies were performed in seven children showing gliosis but no inflammation. Anesthetic barbiturates were used in 14 children with refractory status epilepticus, and immunotherapy in 9. Two children died, eight remained in a state of impaired consciousness, eight developed therapy-refractory epilepsies, two had behavioral disturbances, and two recovered. The lack of evidence for encephalitis suggests another infection-related pathogenesis of this disastrous epileptic encephalopathy. Therefore, we propose the term "febrile infection-related epilepsy syndrome" (FIRES).
脑炎通常被认为是一种疾病，即使在发热性感染后出现癫痫发作且脑脊液中未检测到病原体时也是如此。本回顾性多中心病例系列报告了 22 例先前健康的 3-15 岁儿童（中位数为 6.5 岁），他们在发热后 2-14 天（中位数为 5 天）出现长时间或反复发作的癫痫发作（19 例为呼吸道或非特异性感染）。脑脊液研究显示 2-42 个细胞/微升（中位数为 5 个细胞/微升），无病原体。脑电图显示弥漫性减慢或多灶性放电。神经影像学检查显示 10 例儿童正常。7 例儿童进行了脑活检，显示神经胶质增生但无炎症。14 例难治性癫痫持续状态患儿使用了麻醉巴比妥类药物，9 例患儿使用了免疫疗法。2 例患儿死亡，8 例患儿仍处于意识障碍状态，8 例患儿发展为治疗抵抗性癫痫，2 例患儿出现行为障碍，2 例患儿恢复。缺乏脑炎的证据表明这种灾难性的癫痫性脑病可能与另一种感染相关的发病机制有关。因此，我们提出了“发热感染相关癫痫综合征”（FIRES）这一术语。
