Danesh‐Meyer, Helen V.

AbstractNeuromyelitis optica spectrum disorder (NMOSD) is an antibody‐mediated inflammatory disease of the central nervous system that involves the optic nerves, spinal cord, and often other specific brain regions such as area postrema of the medulla. NMOSD was formerly classified as a variant of multiple sclerosis (MS), given the similar symptomatology and relapsing course but is now considered to have distinct clinical, paraclinical, immunological and prognostic features. The discovery of aquaporin 4 (AQP4) immunoglobulin G (IgG) has improved the ability to diagnose NMOSD. AQP4‐IgG targets the astrocytic AQP4 water channel leading to complement activation and increased blood‐brain barrier permeability. Accurate and early diagnosis is crucial as timely treatment may result in mitigation of long‐term disability. Myelin oligodendrocyte glycoprotein (MOG)‐IgG associated disorder (MOGAD) is a distinct nosologic entity, which has been more recently described. Its clinical spectrum partly overlaps that of seronegative NMOSD and MS. Although it is considered to have fewer relapses and better prognosis than NMOSD, the clinical course and outcome of MOGAD has not been fully characterized.