Frucht Steven
Columbia-Presbyterian Medical Center, The Neurological Institute, New York, New York 1032, USA.
Mov Disord. 2002 May;17(3):609-12. doi: 10.1002/mds.10131.
Rasmussen's encephalitis is a rare autoimmune disorder characterized by intractable epilepsy and progressive hemispheric dysfunction. The disorder usually affects children, although cases have been reported with symptom onset in late adolescence or adulthood. Myoclonus is common in Rasmussen patients, usually occurring as part of epilepsia partialis continua (EPC); however, other hyperkinetic movements are rare. This report documents a 19-year-old woman with Rasmussen's encephalitis whose clinical presentation was dominated by foot dystonia, arm athetosis, and EPC. Intravenous immunoglobulin improved both hyperkinetic movements and EPC, but benefit was transient. The clinical significance and implications of these findings are discussed.
拉斯穆森脑炎是一种罕见的自身免疫性疾病,其特征为顽固性癫痫和进行性半球功能障碍。该疾病通常影响儿童,不过也有在青春期晚期或成年期出现症状的病例报道。肌阵挛在拉斯穆森病患者中很常见,通常作为持续性部分性癫痫(EPC)的一部分出现;然而,其他运动亢进性运动则较为罕见。本报告记录了一名患有拉斯穆森脑炎的19岁女性,其临床表现以足部肌张力障碍、手臂手足徐动症和EPC为主。静脉注射免疫球蛋白改善了运动亢进性运动和EPC,但效果是短暂的。本文讨论了这些发现的临床意义和影响。