Andrews P I, Dichter M A, Berkovic S F, Newton M R, McNamara J O
Department of Pediatrics, Duke university Medical Center, Durham, NC 27710, USA.
Neurology. 1996 Jan;46(1):242-6. doi: 10.1212/wnl.46.1.242.
Rasmussen's encephalitis (RE) is a progressive childhood disorder characterized by intractable focal seizures, hemiplegia, dementia, and inflammatory histopathology. The process is typically limited to one cerebral hemisphere. We report four patients with pathologically confirmed RE who were treated with repeated plasmapheresis. Three patients exhibited repeated, dramatic, transient responses to plasmapheresis, manifested by reduced seizure frequency and improved neurologic function. One patient exhibited marginal improvement after treatment with plasmapheresis. These observations indicate that circulating factors, likely autoantibodies, are pathogenic in at least some patients with RE and suggest that RE is an autoimmune disease. Plasmapheresis may be a useful adjunctive therapy in status epilepticus, and can also aid in assessment of residual function in the diseased hemisphere before surgical resection.
拉斯穆森脑炎（RE）是一种儿童期进行性疾病，其特征为顽固性局灶性癫痫发作、偏瘫、痴呆以及炎症性组织病理学表现。该病变通常局限于一侧大脑半球。我们报告了4例经病理证实为RE的患者，他们接受了重复血浆置换治疗。3例患者对血浆置换表现出反复、显著、短暂的反应，表现为癫痫发作频率降低和神经功能改善。1例患者在接受血浆置换治疗后有轻微改善。这些观察结果表明，循环因子，可能是自身抗体，在至少一些RE患者中具有致病性，并提示RE是一种自身免疫性疾病。血浆置换可能是癫痫持续状态中一种有用的辅助治疗方法，并且在手术切除前还可有助于评估患病半球的残余功能。
