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Introduction: Neuromyelitis optica spectrum disorders (NMOSD), a rare, serious, demyelinating disease of the central nervous system (CNS), is associated with immunoglobulin G (IgG) antibodies targeting aquaporin-4 (AQP4-IgG). This study retrospectively analyzed the clinical features of 67 patients. 49 and 18 of 67 cases (male/female: 11/56) were AQP4-IgG (+) and AQP4-IgG (-), respectively. The initial symptoms were optic neuritis [n = 34, AQP4-IgG (+)/(-): 31/3], myelitis [n = 18, AQP4-IgG(+)/(-): 13/5], co-occurrence of ON and myelitis [n = 15, AQP4-IgG (+)/(-): 5/10]., Conclusions: There was no statistically significant difference between the 2 groups in terms of ages, and magnetic resonance imaging findings, but the patients had a significant difference (P <.05) in sex, the course of disease and Expanded Disability Status Scale (EDSS) scores after drug treatment. Patients with AQP4-IgG (-) are likely to have a better prognosis and a favorable monophasic course.