Kessi Miriam, Liu Fangyun, Zhan Yalan, Tang Yulin, Wu Liwen, Yang Lifen, Zhang Ci-Liu, Yin Fei, Peng Jing
Department of Pediatrics, Xiangya Hospital, Central South University, Changsha, China; Hunan Intellectual and Developmental Disabilities Research Center, Changsha, China; Kilimanjaro Christian Medical University College, Moshi, Tanzania; Mawenzi Regional Referral Hospital, Moshi, Tanzania.
Department of Pediatrics, Xiangya Hospital, Central South University, Changsha, China; Hunan Intellectual and Developmental Disabilities Research Center, Changsha, China.
Seizure. 2020 Jul;79:61-68. doi: 10.1016/j.seizure.2020.04.015. Epub 2020 May 14.
Febrile infection-related epilepsy syndrome is associated with high mortality and morbidity rates. No systematic review of demographics, aetiologies, good treatment options, and causes of deaths has been performed. Thus, we aimed to focus on these factors to provide a structure for patient management and research.
A deep literature search was performed in PubMed and Embase of all years until May 2019.
We retrieved 45 aSrticles: 3 multicentre cohort studies, 13 single-centre cohorts, 1 case series, and 28 case reports. We identified 229 cases: most were from Asia; 53% were males; 11.4% had several types of antibodies, and the most common was anti-glutamate receptor epsilon 2; 30% (69 cases) had good treatment outcomes; 12.2% died; and 56% remained with drug-resistant epilepsies. Univariate analysis revealed a statistically significant association between positive outcomes in Japan and China, the use of the ketogenic diet either acutely or chronically, and the use of steroids acutely or chronically. Taiwan showed a statistically significant association with negative outcomes. Multivariate logistic regression revealed the utilisation of the ketogenic diet in the acute phases (P = 0.008, OR = 3.613) and being in Japan (P = 0.003, OR = 3.146) as independent determinants of positive outcomes. Most of the deaths occurred because of the progress of the disease rather than complications of the drugs.
Asians are more affected and several cases have antibodies. Positive outcomes are associated with being in Japan and the utilisation of the ketogenic diet in the acute phase.
发热感染相关癫痫综合征与高死亡率和发病率相关。目前尚未对其人口统计学、病因、有效治疗方案及死亡原因进行系统综述。因此，我们旨在聚焦这些因素，为患者管理和研究提供一个框架。
截至2019年5月，在PubMed和Embase数据库中对所有年份的文献进行了深入检索。
我们检索到45篇文章：3项多中心队列研究、13项单中心队列研究、1个病例系列和28篇病例报告。我们共纳入229例患者：大多数来自亚洲；53%为男性；11.4%有几种类型的抗体，最常见的是抗谷氨酸受体ε2；30%（69例）治疗效果良好；12.2%死亡；56%仍患有药物难治性癫痫。单因素分析显示，日本和中国的阳性结果、急性或慢性使用生酮饮食以及急性或慢性使用类固醇之间存在统计学显著关联。台湾地区与阴性结果存在统计学显著关联。多因素逻辑回归显示，急性期使用生酮饮食（P = 0.008，OR = 3.613）和来自日本（P = 0.003，OR = 3.146）是阳性结果的独立决定因素。大多数死亡是由于疾病进展而非药物并发症。
亚洲人受影响更大，部分病例存在抗体。阳性结果与来自日本以及急性期使用生酮饮食有关。
