Positive response to immunomodulatory therapy in an adult patient with Rasmussen's encephalitis

Positive response to immunomodulatory therapy in an adult patient with Rasmussen's encephalitis
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DOI:
10.1212/wnl.56.2.248
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发表时间:
2001-01-23
期刊:
影响因子:
9.9
通讯作者:
Avanzini, G
Avanzini, G
中科院分区:
医学1区
文献类型:
--
作者:
Villani, F;Spreafico, R;Avanzini, G

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拉斯穆森脑炎 (RE) 是一种罕见的进行性神经系统疾病,病因不明,通常在儿童期发病。作者描述了一名 45 岁女性,患有成人期进行性失语、右侧偏瘫、严重药物难治性癫痫和左侧大脑半球萎缩。大剂量皮质类固醇和血浆置换术无效。通过人静脉注射免疫球蛋白的高剂量治疗,她的病情有所改善。
Rasmussen's encephalitis (RE) is a rare and progressive neurologic condition of uncertain etiology that typically has a childhood onset. The authors describe a 45-year-old woman with adult-onset progressive aphasia, right hemiparesis, severe drug refractory epilepsy, and left cerebral hemisphere atrophy. High-dose corticosteroids and plasmapheresis were not effective. She improved with high-dose therapy with human IV immunoglobulin.