Bahi-Buisson N, Nabbout R, Plouin P, Bulteau C, Delalande O, Hertz Pannier L, Dulac O, Chiron C
Service de Neurologie et Maladies Métaboliques, Hôpital Necker Enfants Malades, Paris.
Rev Neurol (Paris). 2005 Apr;161(4):395-405. doi: 10.1016/s0035-3787(05)85069-6.
Rasmussen's encephalitis (RE) is a rare inflammatory brain disease mainly affecting children and characterised by intractable epilepsy involving a single hemisphere that undergoes progressive atrophy. RE is characterized by refractory focal seizures, often associated with epilepsia partialis continua, progressive unilateral motor defect, slow EEG activity over the entire contralateral hemisphere, with focal white matter hyperintensity and insular cortical atrophy on neuroimaging. Surgical exclusion of the affected hemisphere is the only treatment that interrupts progression of the disease. Pathogenic concepts have considered viruses, autoimmune antibodies and autoimmune cytotoxic T lymphocytes that might contribute to the initiating or perpetuating events in the central nervous system. Based on these concepts, different therapeutic strategies have been pursued, such as antiviral agents, plasmapheresis, immuno-adsorption, immunosuppression or immunomodulation with intravenous immunoglobulins. However, due to the lack of large studies, to date there is no established therapeutic strategy for this devastating condition. In this review, we give an overview of the current state of immunopathogenic concepts for Rasmussen's encephalitis and discuss the different therapeutic options for future perspectives.
拉斯穆森脑炎（RE）是一种罕见的炎症性脑病，主要影响儿童，其特征为累及单个半球的顽固性癫痫，并伴有进行性萎缩。RE的特点是难治性局灶性癫痫发作，常伴有持续性部分性癫痫，进行性单侧运动缺陷，对侧整个半球脑电图活动缓慢，神经影像学显示局灶性白质高信号和岛叶皮质萎缩。手术切除受累半球是唯一能阻止疾病进展的治疗方法。致病机制涉及病毒、自身免疫抗体和自身免疫性细胞毒性T淋巴细胞，它们可能在中枢神经系统的起始或持续事件中发挥作用。基于这些机制，人们采取了不同的治疗策略，如抗病毒药物、血浆置换、免疫吸附、免疫抑制或静脉注射免疫球蛋白进行免疫调节。然而，由于缺乏大型研究，迄今为止，针对这种毁灭性疾病尚无既定的治疗策略。在本综述中，我们概述了拉斯穆森脑炎免疫致病机制的现状，并讨论了未来不同的治疗选择。
