Abstract

The classic term neuromyelitis optica (NMO) has been replaced by NMO spectrum disorder (NMOSD) as a result of the identification of the diagnostic marker of the disease (IgG-AQP4) and the inclusion of features such as area postrema syndrome or diencephalic disturbances, among others. The article includes the diagnostic criteria for IgG-AQP4 positive and negative NMOSD and describes the treatments used in the acute phase and to prevent relapses (namely, oral steroid-sparing drugs, rituximab, tocilizumab, and drugs specifically developed for the disease). Some of the patients with seronegative IgG-AQP4 NMOSD will show positivity for the IgG-MOG (myelin oligodendrocyte glycoprotein) marker, which can also manifest as recurrent optic neuritis, acute disseminated encephalomyelitis, or conus medullaris syndrome, among others. The possibility of recurrences in some cases of persistently seropositive anti-MOG associated disease prompts the chronic use of immunomodulators in a similar way to TENMO, with the particularity of the usefulness of immunoglobulins in children. Finally, the epidemiological, clinical, paraclinical and therapeutic differences between multiple sclerosis, TENMO and anti-MOG disease are described.

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