Sunwoo Jun-Sang, Lee Soon-Tae, Byun Jung-Ick, Moon Jangsup, Shin Jung-Won, Jeong Da-Eun, Lee Gun-Hee, Jeong Seong Ho, Shin Yong-Won, Jung Keun-Hwa, Lee Doo Young, Jeon Daejong, Jung Ki-Young, Kim Manho, Lee Sang Kun, Chu Kon
Department of Neurology, Laboratory for Neurotherapeutics, Biomedical Research Institute, Seoul National University Hospital, Korea Autoimmune Synaptic and Paraneoplastic Encephalitis Registry (KASPER), Seoul, South Korea.
Department of Neurology, CHA University, CHA Bundang Medical Center, Seongnam, South Korea.
J Neuroimmunol. 2015 Apr 15;281:17-22. doi: 10.1016/j.jneuroim.2015.03.005. Epub 2015 Mar 6.
Contactin-associated protein-like 2 (CASPR2) is one of the target antigens of voltage-gated potassium channels (VGKC) complex antibodies. There has been relatively little information in the literature regarding CASPR2 autoimmunity, especially in Asian population. We investigated the presence of CASPR2 antibodies in patients with presumed autoimmune neurological disorders and described the clinical features, laboratory findings, and responses to immunotherapy. Five patients were identified to be positive for CASPR2 antibodies. The results obtained here suggested that CASPR2 antibodies might be the possible cause of epilepsy even in the absence of typical features of limbic encephalitis and that immunotherapy could provide a favorable outcome.
接触蛋白相关样蛋白2(CASPR2)是电压门控钾通道(VGKC)复合抗体的靶抗原之一。关于CASPR2自身免疫性,尤其是亚洲人群中的相关文献相对较少。我们调查了疑似自身免疫性神经系统疾病患者中CASPR2抗体的存在情况,并描述了其临床特征、实验室检查结果以及免疫治疗反应。有5名患者被确定为CASPR2抗体阳性。此处获得的结果表明,即使没有边缘性脑炎的典型特征,CASPR2抗体也可能是癫痫的潜在病因,并且免疫治疗可能会带来良好的疗效。