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Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory disease of the central nervous system, which is characterized by severe and/or recurrent attacks of myelitis, optic neuritis and area postrema syndrome in most of the patients. NMOSD is strongly associated with serum positivity for aquaporin-4-immunoglobulin G (AQP4-IgG). Although some cases may be diagnosed as NMOSD without AQP4-IgG positivity, additional clinical and neuroimaging features are required by the current international panel for NMOSD diagnostic criteria. These seronegative cases also require the exclusion of an extensive list of alternative diagnoses, including myelin oligodendrocyte glycoprotein (MOG)-IgG-associated disease. We defined and used the term seronegative NMOSD for those patients who fulfil the NMOSD criteria without AQP4-IgG and MOG-IgG seropositivity. The evidence for immune-mediated astrocyte injury caused by both antibody- and complement-dependent cytotoxicity in patients with seronegative NMOSD is not well established compared with those with AQP4-IgG positivity. The therapeutic response to treatments approved for AQP4-IgG-positive NMOSD, such as inebilizumab and satralizumab, also seems to be less clear in seronegative cases, indicating that distinct disease mechanisms may be associated with these patients. [ABSTRACT FROM AUTHOR]

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