Wang Xin, Wang Yisong, Liu Dong, Wang Peigang, Fan Dongying, Guan Yuguang, Li Tianfu, Luan Guoming, An Jing
Department of Microbiology, School of Basic Medical Sciences, Capital Medical University, Beijing, 100069, China.
Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, 100093, China.
Virol Sin. 2017 Oct;32(5):423-430. doi: 10.1007/s12250-017-4058-8. Epub 2017 Oct 30.
Rasmussen's encephalitis (RE) is a rare pediatric neurological disorder, the etiology of which remains unclear. It has been speculated that the immunopathogenesis of RE involves damage to neurons, which eventually leads to the occurrence of RE. Viral infection may be a critical factor in triggering RE immunopathogenesis. In this study, we analyzed the expression of Epstein-Barr virus (EBV) antigens as well as of Toll-like receptor 3 (TLR3), TLR9, and downstream adapter TIRdomain-containing adapter-inducing interferon-β (TRIF) in the brain tissues of 26 patients with RE and 16 control individuals using immunohistochemistry (IHC). In the RE group, EBV antigens were detected in 53% of individuals at various expression levels. In contrast, there was no detectable EBV antigen expression in control brain tissues. Moreover, we found marked increases in the expression of TLR3, TLR9, and TRIF in the brain tissues of RE patients compared with levels in the control group. Furthermore, among RE cases, EBV expression and high TLR3 expression were associated with more severe brain atrophy. Our results suggest that the elevated expression of EBV and TLRs may be involved in RE occurrence through the activation of downstream molecules.
拉斯穆森脑炎（RE）是一种罕见的儿科神经系统疾病，其病因尚不清楚。据推测，RE的免疫发病机制涉及神经元损伤，最终导致RE的发生。病毒感染可能是触发RE免疫发病机制的关键因素。在本研究中，我们采用免疫组织化学（IHC）分析了26例RE患者和16例对照个体脑组织中爱泼斯坦-巴尔病毒（EBV）抗原以及Toll样受体3（TLR3）、TLR9和下游衔接蛋白含TIR结构域的衔接蛋白诱导干扰素-β（TRIF）的表达。在RE组中，53%的个体在不同表达水平检测到EBV抗原。相比之下，对照脑组织中未检测到EBV抗原表达。此外，我们发现与对照组相比，RE患者脑组织中TLR3、TLR9和TRIF的表达显著增加。此外，在RE病例中，EBV表达和高TLR3表达与更严重的脑萎缩有关。我们的结果表明，EBV和TLRs的表达升高可能通过下游分子的激活参与RE的发生。
