Particular attention will be paid to the unique obstacles we face when predicting the risk of relapse and the important implications of such challenges when planning treatment protocols. We will review data on the acute care of a demyelinating attack in addition to treatment of patient with relapsing diseases. In this review, we will focus on pediatric-onset MOG-Ab-associated disease, with specific attention to challenging clinical scenarios. Emerging consensus supports distinction of treatment strategies from those typically used for relapsing remitting MS, and several groups debate whether to follow treatment protocols akin to those for AQP4-Ab NMOSD. Treatment of patients with MOG-positive demyelination includes management of acute relapses and chronic immunotherapy for those with relapsing MOG disease. Features of such patients that render them “atypical” for MS will be presented. Ī proportion of patients with MOG-Abs meet the McDonald 2017 criteria for a diagnosis of MS at onset and can experience relapses typical of MS posing significant diagnostic and treatment challenges. Published cohort studies of adults and children with NMOSD demonstrate that MOG-Abs associated with younger patients (particularly children) are frequently in males (and do not demonstrate the strong female preponderance seen in AQP4-Ab NMOSD), and despite the relapsing disease course, patients demonstrate good recovery from the acute relapses and the overall visual and motor outcome is better compared with AQP4-Ab-positive patients. Identification of MOG-Ab at the time of incident ADS, however, does not predict risk of relapse, as the majority of MOG-Ab-positive children with acute disseminated encephalomyelitis (ADEM) or isolated optic neuritis (with normal brain MRI) experience a monophasic disease course. The presence of MOG-Ab identifies a subset of adults and children meeting the clinical and imaging criteria for neuromyelitis optica spectrum disorder (NMOSD) without antibodies to aquaporin-4. Īntibodies to MOG (MOG-Abs) have been detected in 30–50% of children at first presentation of acquired demyelinating syndrome (ADS), with two studies from the UK/France and the Netherlands suggesting that MOG-Abs identified at onset are associated with a non-MS disease course. In animal models, antibodies identified following MOG induction appear to mediate or contribute directly to demyelination. Although MOG represents only a minor component (0.05%) of the myelin sheath, its location on the outermost lamellae and on the cell surface of oligodendrocytes makes it highly immunogenic and available for antibody binding. The myelin protein, myelin oligodendrocyte glycoprotein (MOG), is exclusively expressed in the central nervous system (CNS).
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