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Although some of their characteristics overlap, neuromyelitis optica spectrum disorder (NMOSD) is distinct from multiple sclerosis (MS) in many clinical, laboratory, and neuroimaging features, including its strong association with a pathogenic autoantibody biomarker, AQP4-IgG.1 The specificity of AQP4-IgG was leveraged by its incorporation into revised diagnostic criteria in 2006.2 Subsequently, a number of additional clinical phenotypes and magnetic resonance imaging (MRI) patterns were noted to occur in AQP4-IgG-seropositive neuromyelitis optica (NMO) patients who fulfilled 2006 criteria. These presentations (notably, brain involvement and “NMO-typical” MRI lesion patterns) were proposed to be part of an umbrella term “NMO spectrum disorders” in 2007.3

Neuromyelitis optica spectrum disorder diagnostic criteria: Sensitivity and specificity are both important