Abstract: Neuromyelitis optic spectrum disorder (NMOSD) is an inflammatory, demyelinating syndrome of the central nervous system that is characterized by severe attacks of optic neuritis and myelitis. Most patients follow a course of early incremental disability due to frequent and severe relapses. And there are no effective drugs to avoid its recurrence. Monoclonal antibodies have made remarkable breakthrough in the treatment of NMOSD. Recent studies have provided effective evidence to support the efficacy and safety of monoclonal antibodies in the treatment of NMOSD. This paper reviews the research progress of targeted drugs to interleukin-6 receptor, B cell, Complement 5, and potential treatment strategies in AQP4-IgG-seronegative NMOSD.