Abstract: Neuromyelitis optica spectrum disorders (NMOSD) are a range of central nervous system(CNS)inflammatory demyelinating diseases which mainly involve the optic nerve and spinal cord. Antibody against aquaporin 4(AQP 4), a channel protein widely distributed in the astrocyte foot process, is found to be the cause of NMOSD. The combination of AQP4 antibody with antigen can cause complement activation, up-regulation of chemokines and inflammatory cytokines in B cells and T cells, and further promote inflammatory reaction. Interleukin-6 can promote B cell activation, induce Th cell polarization and AQP4 antibody production, which increase blood-brain barrier permeability and promote inflammatory cell infiltration in the central nervous system. Overgrowth of Clostridium perfringens (CPs) promotes cell differentiation into T helper cells 17, and CPS is involved in the pathogenesis of NMOSD by affecting the balance between Th17 and regulatory T cells(Treg). This paper reviewed the recent advances of pathogenesis of NMOSD, aiming to provide reference for the diagnosis and treatment of NMOSD.