Cheli Marta, Dinoto Alessandro, Tommasini Valentina, Ajčević Miloš, Stella Giulio, Catalan Mauro, Stokelj David, Sartori Arianna, Manganotti Paolo
Clinical Unit of Neurology, Department of Medicine, Surgery and Health Sciences, Cattinara University Hospital ASUGI, University of Trieste, Strada di Fiume, 447 - 34149 Trieste, Italy.
Department of Engineering and Architecture, University of Trieste, Trieste, Italy.
Epileptic Disord. 2020 Oct 1;22(5):678-682. doi: 10.1684/epd.2020.1213.
LGI1 encephalitis is an autoimmune disorder characterized by cognitive symptoms and seizures, which rarely respond to common antiepileptic drugs (AEDs). Rituximab (RTX) is a CD-20-depleting monoclonal antibody which has been used for the treatment of LGI1 encephalitis, however, its efficacy remains controversial. A 54-year-old woman came to our attention due to memory loss and gambling. Brain MRI revealed areas of bilateral hippocampal hyperintensity and LGI1 antibodies were found in both serum and cerebrospinal fluid. Immunotherapy with steroids was started, followed by intravenous immunoglobulins with partial improvement. The patient developed multiple generalized tonic-clonic seizures. She was then administered intravenous rituximab with significant improvement for both cognitive symptoms and seizure control. High-density EEG was recorded before treatment, seven days after the first dose and seven days after the second dose. Topoplot and power spectrum analysis were performed for each recording. Interictal epileptiform discharges, as well as theta power bands, were significantly reduced after each dose, while topoplot analysis showed reduced spreading over posterior and frontal electrodes for interictal epileptiform discharges of temporal origin. Our experience indicates that rituximab is a valid treatment for LGI1 encephalitis, demonstrating efficacy for both cognitive symptoms and seizure control. High-density EEG could represent a novel, safe and reproducible method to study epileptogenesis in autoimmune limbic encephalitis.
LGI1 脑炎是一种自身免疫性疾病,其特征为认知症状和癫痫发作,对常用抗癫痫药物(AEDs)反应不佳。利妥昔单抗(RTX)是一种可清除 CD-20 的单克隆抗体,已被用于治疗 LGI1 脑炎,然而其疗效仍存在争议。一名 54 岁女性因记忆力减退和赌博行为引起我们的关注。脑部 MRI 显示双侧海马区高信号,血清和脑脊液中均发现 LGI1 抗体。开始使用类固醇进行免疫治疗,随后静脉注射免疫球蛋白,症状有部分改善。患者出现多次全身性强直阵挛发作。随后给她静脉注射利妥昔单抗,认知症状和癫痫控制均有显著改善。在治疗前、首次给药后 7 天和第二次给药后 7 天记录高密度脑电图。对每次记录进行拓扑图和功率谱分析。每次给药后,发作间期癫痫样放电以及 θ 功率带均显著减少,而拓扑图分析显示,起源于颞叶的发作间期癫痫样放电在后部和额叶电极上的扩散减少。我们的经验表明,利妥昔单抗是治疗 LGI1 脑炎的有效药物,对认知症状和癫痫控制均有疗效。高密度脑电图可能是研究自身免疫性边缘性脑炎癫痫发生机制的一种新颖、安全且可重复的方法。