电压门控钾通道自身抗体相关性边缘性脑炎的研究进展

摘要

边缘性脑炎(LE)是指选择性地累及边缘性结构的一类中枢神经系统炎性疾病.电压门控钾通道(VGKC)为镶嵌在中枢和周围神经系统神经元膜上的糖蛋白,是神经系统信号传导的组成元件,在维持静息膜电位和神经元动作电位中发挥着关键作用.VGKC自身抗体相关性LE是常见的自身免疫性神经系统疾病,患者体内能与VGKC发生免疫共沉淀的抗体主要是富亮氨酸胶质瘤失活1蛋白(LGI1)和接触蛋白相关样蛋白2(Caspr2),故其可分为抗LGI1抗体阳性LE、抗Caspr2抗体阳性LE、LGI1和Caspr2抗体阴性LE.本文对VGKC自身抗体相关性LE患者的临床表现、诊断及治疗方法进行了综述,以期提高VGKC自身抗体相关性LE临床诊治效果及改善患者预后.%Limbic encephalitis(LE) means a kind of central nervous system inflammatory disease that selectively involved marginal structure.Voltage-gated kalium channel(VGKC),a kind of glycoprotein that insetting in central and peripheral nervous system neuronal membranes,is one of constituent elements of nervous system signaling pathway,play an pivotal role in maintaining resting membrane potential and neuronal action potential.VGKC autoantibody related LE is one of common autoimmune nervous system diseases,LGI1 and Caspr2 are the major proteins of VGKC that may serve as antibodies and participate in the co-immunoprecipitation,thus VGKC autoantibody related LE can be divided into LGI1 antibody positive LE,Caspr2 antibody positive LE,LGI1- and Caspr2-antibody negative LE.This paper reviewed the clinical features,diagnosis and therapeutic methods of VGKC autoantibody related LE,to improve the clinical diagnostic and therapeutic effect,and the prognosis.