Du Yi, Li Kaijun, Liu Wei, Song Ruitong, Luo Meifeng, He Jianfeng, Xu Xiaoyu, Qu Xiaosheng
Department of Ophthalmology, the First Affiliated Hospital of Guangxi Medical University, 530021, Nanning,China.
Doheny Eye Institute, Department of Ophthalmology, University of California, Los Angeles, CA 90033,United States.
Curr Pharm Des. 2022;28(4):272-279. doi: 10.2174/1381612827666210329101335.
Neuromyelitis optica spectrum disorder (NMOSD) is an acute or subacute demyelinating disease that affects mainly the optic nerve and spinal cord. A major proportion of NMOSD cases have a relationship with autoimmunity to aquaporin 4 (AQP4) found on the central nervous system. NMOSD can occur repeatedly, causing symptoms such as decreased vision and weakness of limbs. The main goal of the current therapy is to relieve acute symptoms and prevent recurrence of the disease. Without timely and appropriate treatment, the recurrence and disability rates are high. In the present work, we review recent advances in the diagnosis and treatment of patients with NMOSD, as well as the pathogenesis and mechanisms of AQP4-IgG-seropositive NMOSD.
视神经脊髓炎谱系障碍(NMOSD)是一种主要影响视神经和脊髓的急性或亚急性脱髓鞘疾病。大部分NMOSD病例与针对中枢神经系统中发现的水通道蛋白4(AQP4)的自身免疫有关。NMOSD可反复发生,导致视力下降和肢体无力等症状。当前治疗的主要目标是缓解急性症状并预防疾病复发。若不及时进行适当治疗,复发率和致残率都很高。在本研究中,我们综述了NMOSD患者诊断和治疗的最新进展,以及AQP4-IgG血清阳性NMOSD的发病机制。