摘要： 视神经脊髓炎是主要累及视神经和脊髓的自身免疫性炎性脱髓鞘疾病。自2004 年在视神经脊髓炎患者血清中发现特异性抗体NMO-IgG 以来，相继发现其作用靶点是广泛分布于视神经、脊髓、脑室周围区域的水通道蛋白4（AQP4），故亦称之为AQP4 抗体。NMO-IgG 对视神经脊髓炎的诊断与鉴别诊断，以及对疾病活动性、药物疗效和预后评价均具有重要临床意义。近年基于组织、细胞和蛋白质测定的多种方法应用于血清或脑脊液NMO-IgG 检测，本文拟对不同检测方法之进展和临床意义进行概述。

关键词: 视神经脊髓炎, 水通道蛋白质4, 综述

Abstract: Neuromyelitis optica (NMO) is an inflammatory demyelinating disease of the central nervous system (CNS) of autoimmune etiology which predominantly affects the optic nerves and spinal cord. In 2004, a highly specific serum antibody, NMO-IgG, was found in the sera of NMO patients. Subsequently, the target antigen of NMO-IgG was identified as aquaporin 4 (AQP4), a water channel densely expressed in optic nerves, spinal cord and area around cerebral ventricles. NMO-IgG/AQP4 antibody has demonstrated extreme importance for the diagnosis and differential diagnosis, the evaluation of disease activity, therapeutic effect and prognosis of NMO. In recent years, different techniques have been used to examine NMO-IgG in serum and cerebrospinal fluid, including tissue-based, cell-based and protein-based assays. In this review, the authors give an overview of the tests currently available for the detection of NMO-IgG and their clinical significance.

Key words: Neuromyelitis optica, Aquaporin 4, Review