Brainstem demyelination could also occur in MOGAD however, area postrema syndrome or internuclear ophthalmoplegia is associated with NMOSD or MS, respectively ( 5, 67). MOGAD most commonly presents as bilateral optic neuritis, transverse myelitis, ADEM, or, less commonly, cerebral cortical encephalitis. Distinctive features of MOGADĭistinctive clinical, radiological, and histopathological features and laboratory findings of MOGAD are summarized below ( Table 1). progressive disease) and MOG as the autoimmune trigger ( 64– 66). For this purpose, we will use evidence from human and animal studies such as MOG-induced experimental autoimmune encephalomyelitis (EAE) models, as some of these models closely resemble MOGAD compared to MS due to relapsing and remitting course (vs. This review will highlight major distinctive clinical, radiological, and histopathological features of MOGAD and summarize how different immune compartments contribute disease pathogenesis. How humoral immunity and cellular immunity cooperate in MOGAD pathogenesis is an intriguing subject. Neuroinflammatory biomarkers (such as MBP, sNFL, GFAP, Tau) in MOGAD support that most axonal damage happens in the initial attack, whereas relapses are associated with increased myelin damage.Īutoimmunity in MOGAD starts at the periphery by activation of T cells and production of autoantibodies and eventually transfer of these immune mediators into the CNS ( 5, 38). Stimulation of peripheral MOG-specific B cells through TLR stimulation or T follicular helper cells might help differentiate MOG antibody-producing plasma cells in the peripheral blood. MOG antibodies potentially contribute to pathology by opsonizing MOG, complement activation, and antibody-dependent cellular cytotoxicity. Granulocytes, macrophages/microglia, and activated complement are also found in the lesions, which could contribute to demyelination during acute relapses. CD4+ T cells, unlike CD8+ T cells in MS, are the dominant T cell type found in lesion histology. Initial activation of T cells in the periphery is followed by reactivation in the subarachnoid/perivascular spaces by MOG-laden antigen-presenting cells and inflammatory CSF milieu, which enables T cells to infiltrate CNS parenchyma. MOGAD lesions show a perivenous confluent pattern around the small veins, lacking the radiological central vein sign. MOGAD pathophysiology is driven by acute attacks during which T cells and MOG antibodies cross blood brain barrier (BBB). Interestingly, pediatric MOGAD is not more aggressive than adult-onset MOGAD, unlike in multiple sclerosis (MS), where annualized relapse rates are three times higher in pediatric-onset MS. In addition to distinct clinical, radiological, and immunological features, the infectious prodrome is more commonly reported in MOGAD (37–70%) than NMOSD (15–35%). Myelin Oligodendrocyte Glycoprotein Antibody Disease (MOGAD) is a spectrum of diseases, including optic neuritis, transverse myelitis, acute disseminated encephalomyelitis, and cerebral cortical encephalitis. 2Department of Neurology, Brigham and Women's Hospital, Ann Romney Center for Neurologic Diseases, Boston, MA, United States.1Harvard Medical School, Boston, MA, United States.
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