Aradillas Enrique, Schwartzman Robert J
Department of Neurology, Drexel University College of Medicine, Philadelphia, PA 19102-1192, USA.
Arch Neurol. 2011 Apr;68(4):529-32. doi: 10.1001/archneurol.2010.317. Epub 2010 Dec 13.
To describe the first case (to our knowledge) of voltage-gated potassium channel-complex protein antibody encephalitis with kinesigenic dyskinesia and cramp-fasciculation syndrome.
Case report.
Hospitalized care.
A 38-year-old man with a history of bronchial asthma, eczema, vitiligo, and immune complex mesangiopathic glomerulonephritis presented with abnormal movements.
Clinical examination, magnetic resonance imaging, single-photon emission computed tomography, electromyography and nerve conduction studies, video-electroencephalographic monitoring, plasmapheresis exchange therapy, and intravenous immunoglobulin administration.
Clinical examination revealed paroxysmal kinesigenic dyskinesia and fasciculations. Magnetic resonance imaging of the brain revealed a left caudate and left putamen increased signal lesion on T2-weighted and fluid-attenuated inversion recovery sequences as well as increased flow in the same region on single-photon emission computed tomographic scans. Electromyography and nerve conduction studies revealed significant afterdischarges, cramp potentials, and continuous motor activity. The video-electroencephalographic monitoring revealed no epileptiform discharges. The patient dramatically improved after 5 plasmapheresis exchange treatments and a course of intravenous immunoglobulin at 2 gm/kg over 5 divided doses.
To our knowledge, this is the first report of paroxysmal kinesigenic dyskinesia with voltage-gated potassium channel-complex protein antibody encephalitis associated with the cramp fasciculation syndrome.
描述首例(据我们所知)伴有运动诱发性运动障碍和肌阵挛-束颤综合征的电压门控钾通道复合蛋白抗体脑炎病例。
病例报告。
住院治疗。
一名38岁男性,有支气管哮喘、湿疹、白癜风和免疫复合物系膜增生性肾小球肾炎病史,出现异常运动。
临床检查、磁共振成像、单光子发射计算机断层扫描、肌电图和神经传导研究、视频脑电图监测、血浆置换疗法和静脉注射免疫球蛋白。
临床检查发现阵发性运动诱发性运动障碍和肌束震颤。脑部磁共振成像显示,在T2加权和液体衰减反转恢复序列上,左侧尾状核和左侧壳核信号增强病变,以及在单光子发射计算机断层扫描中同一区域血流增加。肌电图和神经传导研究显示明显的后放电、肌痉挛电位和持续运动活动。视频脑电图监测未发现癫痫样放电。患者在接受5次血浆置换治疗和一个疗程的静脉注射免疫球蛋白(2克/千克,分5次给药)后显著改善。
据我们所知,这是首例伴有运动诱发性运动障碍的电压门控钾通道复合蛋白抗体脑炎与肌阵挛束颤综合征相关的报告。