Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Italy.
Pediatric Clinic IRCCS Policlinico San Matteo Foundation, Pavia, Italy.
Seizure. 2020 Oct;81:76-83. doi: 10.1016/j.seizure.2020.07.023. Epub 2020 Jul 25.
Rasmussen encephalitis (RE) is a unilateral hemispheric encephalitis whose main clinical features include refractory focal epilepsy or epilepsia partialis continua, hemiparesis, and progressive cognitive decline. Despite the autoimmune pathogenesis of RE, the only definitive therapeutic option is currently represented by surgery. We review the clinical features, the immune pathogenesis, and the available therapeutic options for RE, with special focus on immunosuppressive agents. The research includes systematic reviews, meta-analyses, observational studies, clinical trials, cases series and reports, until 2020. The use of immunosuppressive agents in RE is supported by the evidence of an autoimmune involvement of the central nervous system in this condition. Although often insufficient to modify the disease course and to achieve symptomatic control, immune therapy can be effective in patients with slow disease progression or in patients in which surgery is not applicable. Moreover, the documentation of T-cell involvement in the pathogenesis of RE, with a specific cytokine pattern, opens a window of opportunity for the use of T-targeted therapies and biologic drugs (i.e. anti-TNFα agents) in the treatment of this disease.
拉森姆森脑炎（RE）是一种单侧大脑半球脑炎，其主要临床特征包括难治性局灶性癫痫或部分性癫痫持续状态、偏瘫和进行性认知功能下降。尽管 RE 的发病机制是自身免疫性的，但目前唯一明确的治疗选择仍是手术。我们回顾了 RE 的临床特征、免疫发病机制和现有的治疗选择，特别关注了免疫抑制药物。研究包括系统评价、荟萃分析、观察性研究、临床试验、病例系列和报告，截至 2020 年。免疫抑制药物在 RE 中的应用得到了中枢神经系统自身免疫参与这一疾病的证据支持。尽管免疫治疗通常不足以改变疾病进程和实现症状控制，但对于疾病进展缓慢或手术不适宜的患者，免疫治疗可能有效。此外，T 细胞参与 RE 发病机制的证据，以及特定的细胞因子模式，为 T 细胞靶向治疗和生物药物（即抗 TNFα 药物）在治疗这种疾病中的应用开辟了机会。
