Bien C G, Granata T, Antozzi C, Cross J H, Dulac O, Kurthen M, Lassmann H, Mantegazza R, Villemure J-G, Spreafico R, Elger C E
Department of Epileptology, University of Bonn, Sigmund-Freud-Str. 25, 53105 Bonn, Germany.
Brain. 2005 Mar;128(Pt 3):454-71. doi: 10.1093/brain/awh415. Epub 2005 Feb 2.
Rasmussen encephalitis (RE) is a rare but severe immune-mediated brain disorder leading to unilateral hemispheric atrophy, associated progressive neurological dysfunction and intractable seizures. Recent data on the pathogenesis of the disease, its clinical and paraclinical presentation, and therapeutic approaches are summarized. Based on these data, we propose formal diagnostic criteria and a therapeutic pathway for the management of RE patients.
拉斯穆森脑炎(RE)是一种罕见但严重的免疫介导性脑部疾病,可导致单侧半球萎缩、相关的进行性神经功能障碍和难治性癫痫发作。本文总结了该疾病发病机制、临床和辅助检查表现以及治疗方法的最新数据。基于这些数据,我们提出了针对拉斯穆森脑炎患者管理的正式诊断标准和治疗途径。