![]() Nine patients tested positive for MOG of whom 6 patients received an initial diagnosis of ADEM, encephalitis or meningitis. A second Japanese case series studied MOG seropositivity in 17 children presenting with inflammatory demyelinating disease. In a small Japanese cohort of 4 cases with MOG antibody encephalitis, all were male with a median age of 37 years. In a related Chinese cohort of AQP4 seropositive NMOSD patients, only 3.6% of patients experienced an attack of encephalitis, all of whom were females in their late 30s. Ten were males and 8 were females the median age of onset was 22 years. In a Chinese study published in 2018, 20.7% of MOG positive patients had a typical presentation of encephalitis at some point in their disease course. ADEM tends to strike children much more than adults whereas MOG is found in both children and adults alike. MS patients are slightly older than MOG patients on average and share the propensity for Caucasian descent but women with MS outnumber men by 3:1. This is different from AQP4 NMOSD in which the average age of onset is in the late 30s/early 40s, race is overrepresented by non-Caucasians and females dominate up to 9:1. Recent data suggest that MOG antibody disease is generally a condition of younger Caucasians with less female predominance compared to other autoimmune diseases of the central nervous system (CNS). These studies contribute to the emerging consensus that MOG antibody disease is likely to be a separate immunopathological entity with a distinctive clinical signature. Most of these cases were previously diagnosed as ADEM or recurrent ADEM but with widespread availability of MOG antibody testing since 2015, several case series and reports have been published on cases of encephalitis associated with MOG antibody. In this review, we focus on encephalitis associated with the MOG antibody. MOG has been ironically unique in that it shares features of NMOSD, MS and ADEM with only a serological antibody to distinguish itself. Unlike ADEM which is classically monophasic, seropositivity of the MOG antibody portends a high likelihood of relapse. When MOG antibody disease involves the brain, the phenotype is similar to acute disseminated encephalomyelitis (ADEM) with alterations in mental status and good outcomes after treatment with corticosteroids and intravenous immunoglobulins. Thus, immune mediated attacks against MOG appear to be more demyelinating compared with AQP4 NMOSD, and therefore MOG is closer to multiple sclerosis (MS) in terms of its immunopathological features. However, unlike AQP4 which is an astrocytic protein, MOG is expressed on the outer surface of CNS myelin. It accounts for up to 40% of NMOSD patients who are seronegative for the aquaporin-4 (AQP4) antibody. Clinically, MOG antibody disease bears resemblance to neuromyelitis optica spectrum disorder (NMOSD) in the localization of inflammatory attacks within the central nervous system. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.MOG antibody disease refers to a relatively new spectrum of autoimmune disorder with antibodies against the myelin oligodendrocyte glycoprotein (MOG) predominantly involving the optic nerve and spinal cord leading to vision loss and paralysis. Inflammatory demyelinating diseases (IDDs) MOG-IgG Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) Neuro-ophthalmology Neurology Ophthalmology. Key challenges to the current understanding of MOGAD are also highlighted, including uncertainty regarding the specificity and pathogenicity of MOG autoantibodies, the need to identify immunopathologic targets for future therapies, the quest to validate biomarkers that facilitate diagnosis and detect disease activity, and the importance of deciphering which patients with MOGAD require long-term immunotherapy. In this review, cardinal clinical features of MOGAD are discussed. For this reason, possible alternative diagnoses need to be considered, and low MOG-IgG titers need to be carefully weighted. Over the last several years, access to cell-based assay (CBA) techniques has improved diagnostic accuracy, yet the positive predictive value of serum MOG-IgG values varies with the prevalence of MOGAD in any given patient population. Seropositivity status for MOG-IgG autoantibodies is important for diagnosing MOGAD, but only in the context of robust clinical characterization and cautious interpretation of neuroimaging. New diagnostic criteria for myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) have recently been proposed, distinguishing this syndrome from other inflammatory diseases of the central nervous system. ![]()
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