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The French term “neuro-myélite optique aiguë,” which may be translated as “neuromyelitis optica acuta” was first used by Devic in 1894 ( 1, 2). Neuromyelitis optica spectrum disorders (NMOSD) are rare chronic inflammatory central nervous system diseases distinct from multiple sclerosis (MS). We discuss therapeutic options of acute attacks as well as longterm immunosuppressive treatment, including azathioprine, rituximab, and immunoglobulins. These include antibody and further laboratory testing, MR imaging and optical coherence tomography. Here we give an overview on current recommendations concerning diagnosis of NMOSD and MOG-EM. These clinical syndromes are now frequently referred to as “MOG-encephalomyelitis” (MOG-EM). In some patients with symptoms of NMOSD, no AQP4-Abs but Abs against myelin-oligodendrocyte-glycoprotein (MOG) are detectable. Serum antibodies (Abs) against the aquaporin-4 water channel lead to recurrent attacks of optic neuritis, myelitis and/or brainstem syndromes. Neuromyelitis optica spectrum disorders (NMOSD) are autoantibody mediated chronic inflammatory diseases. 4Experimental and Clinical Research Center, Max Delbrueck Center for Molecular Medicine and Charité – Universitätsmedizin Berlin, Berlin, Germany.3Department of Neuroradiology, Charité – Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany.2Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.1NeuroCure Clinical Research Center, Charité – Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany.Nadja Borisow 1 *, Masahiro Mori 2, Satoshi Kuwabara 2, Michael Scheel 1,3 and Friedemann Paul 1,4