5 вЂ“ 7 For this reason, MOG-IgG antibodies (MOG-IgG) were extensively studied in the last two decades in different acquired demyelinating syndromes (ADSs). 3, 4 Knowledge about the biological role of MOG and its isoforms is limited however, the encephalitogenic potential of MOG that is eliciting demyelinating immune responses has been demonstrated in numerous experimental models. The differences in the C-terminal amino acids are the basis to distinguish О± or ОІ isoforms of MOG. 1, 2 Multiple isoforms of MOG exist that have identical extracellular immunoglobulin (Ig) domains, but differentially spliced intracellular C-termini. Myelin oligodendrocyte glycoprotein (MOG) is a highly conserved protein that is exclusively expressed in oligodendrocytes in the central nervous system (CNS). In this review, we provide an overview of current knowledge of MOG-IgG associated disorders, describe the clinical presentations identified, highlight differences from neuromyelitis optica spectrum disorders and multiple sclerosis, summarize clinical outcome and concepts of immune treatment, depict the underlying mechanisms of antibody pathogenicity and provide the methodological essentials of MOG-IgG assays. In recent studies, phenotype of MOG-IgG associated disorders has further broadened with the description of cases of brainstem encephalitis, encephalitis with seizures and overlap syndromes with other types of autoimmune encephalitis. MOG-IgG associated disorders comprise a wide spectrum of syndromes ranging from acute disseminated encephalomyelitis predominantly in children to optic neuritis or myelitis mostly in adults. The meticulous clinical description of patients with MOG IgG antibodies (MOG-IgG) has been achieved by development and use of highly specific cell-based assays. In the past few years, acquired demyelinating syndromes of the central nervous system associated with antibodies against myelin oligodendrocyte glycoprotein (MOG) have evolved into a new inflammatory disease entity distinct from neuromyelitis optica spectrum disorders or multiple sclerosis.

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