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Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and neuromyelitis optica spectrum disorders (NMOSD) are two rare autoimmune inflammatory demyelinating diseases involving the central nervous system, which are often seen with combined involvement of the optic nerve and spinal cord. MOGAD can be confused with multiple sclerosis or NMOSD, due to its clinical presentation that may be similar and its characteristic to progress with habitual attacks. Although the clinical course of the above-mentioned three diseases is similar, their diagnosis and management are different.

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While advancements in the classification of acquired demyelinating syndromes have significantly benefited children with this condition, some cases present with overlapping features, posing diagnostic challenges. We describe an Asian girl of early childhood age with acute visual loss. Examination revealed right optic neuritis, left optic nerve atrophy and demyelinating lesions in the juxtacortical brain parenchyma.

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Some patients with neuromyelitis optica spectrum disorder (NMOSD)-like symptoms test negative for anti-aquaporin-4 (anti-AQP4) antibodies. Among them, a subset has antibodies targeting myelin oligodendrocyte glycoprotein (MOG), a condition now termed MOG antibody-associated disease (MOGAD). MOGAD shares features with NMOSD, like optic neuritis and myelitis, but differs in pathophysiology, clinical presentation, imaging findings, and biomarkers.

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Antimyelin-associated glycoprotein (MAG) neuropathy is a rare autoimmune demyelinating peripheral neuropathy caused by IgM autoantibodies targeting MAG. The typical presentation is that of a slowly progressive, distal, length-dependent, predominantly sensory, sometimes ataxic neuropathy, frequently accompanied by upper limb tremor. Distal motor weakness may subsequently occur.

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Background: Few studies have examined B cells among patients with anti-myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD), including brain pathology.

Objective: To describe cases of tumefactive MOGAD with B-cell dominant central nervous system (CNS) infiltration.

Methods: In this study, we reviewed three cases with clinical and brain histopathological features with tumefactive MOGAD.

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