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Recent advances in the understanding of neuromyelitis optica spectrum of disorders (NMOSD) have expanded. Diagnostic criteria have changed over the years. The clinical spectrum of disease manifestations are now understood to include sites outside the spinal cord and optic nerve. A variety of autoimmune diseases may coexist with this disorder. Non neurological manifestations have been recently reported. Novel biomarkers other than aquoporin 4 Immunoglobulin G (anti AQP4-IgG) have been discovered which may have clinical relevance. In particul myelin associated oligoglycoprotein antibody (MOG-Ab) associated NMOSD may be relatively benign. This update describes some of these new findings highlighting the clinical manifestations, biomarkers associated with the disease and magnetic resonance imaging characteristics of brain and spinal cord.