reported four patients who they described as having MOG antibody-positive, benign, unilateral, cerebral cortical encephalitis with epilepsy, indicating a novel phenotype of MOGAD ( 11). The focus on ADEM as the primary encephalitic manifestation of MOGAD was highlighted in early recommendations on anti-MOG testing and diagnosis, which did not emphasize any other cerebral disease presentations ( 9, 10). In particular, mention of encephalitis in MOGAD has historically been in reference to acute disseminated encephalomyelitis (ADEM), which is a multifocal inflammatory demyelinating disease that presents with encephalopathy and large T2-hyperintense lesions predominantly involving the cerebral white matter ( 8). Classical manifestations of MOG antibody-associated disease (MOGAD) that were initially described in the literature included optic neuritis, myelitis, brainstem demyelination and encephalitis. Over the last decade, antibodies against myelin oligodendrocyte glycoprotein (MOG) have emerged as a biomarker of inflammatory demyelinating disease that is distinct from multiple sclerosis ( 1– 7). Subsequently, anti-MOG positivity in patients with a variety of cortical and meningeal disease presentations has been reported, indicating a broader spectrum of meningo-cortical manifestations in MOGAD that we review herein. In 2019, we systematically reviewed the literature to better characterize this unique syndrome, which we referred to as unilateral cortical FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES). described four patients with seizures, unilateral cortical hyperintensities on brain magnetic resonance imaging T2-fluid-attenuated inversion recovery sequences, and anti-MOG positivity, indicating a potentially novel form of encephalitis in MOGAD. Initial manifestations of MOGAD that were reported in the literature included optic neuritis, myelitis, brainstem demyelination and encephalitis, with emphasis placed on acute disseminated encephalomyelitis (ADEM) as the primary encephalitic presentation. Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an inflammatory demyelinating disease that is distinct from multiple sclerosis. 3Department of Medical Imaging, Western University, London Health Sciences Centre, London, ON, Canada.2Department of Pathology and Laboratory Medicine, Western University, London Health Sciences Centre, London, ON, Canada.1Department of Clinical Neurological Sciences, Western University, London Health Sciences Centre, London, ON, Canada.eCollection 2018.Adrian Budhram 1,2 * Ario Mirian 1 Manas Sharma 1,3 Defining distinct features of anti-MOG antibody associated central nervous system demyelination. (4) Weber MS, Derfuss T, Metz I, Brück W. MOG-IgG-Associated Optic Neuritis, Encephalitis, and Myelitis: Lessons Learned From Neuromyelitis Optica Spectrum Disorder. (3) Dos Passos GR, Oliveira LM, da Costa BK, et al. Diagnosis and treatment of anti-myelin oligodendrocyte glycoprotein antibody positive optic neuritis. Comparison of myelin oligodendrocyte glycoprotein (MOG)-antibody disease and AQP4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) when they co-exist with anti-NMDA (N-methyl-D-aspartate) receptor encephalitis. 4 Furthermore, those with MOG antibody disease seem to be less likely to have other autoimmune disorders (such as rheumatoid arthritis, Hashimoto’s thyroiditis, etc.) than those with AQP-4 positive NMOSD. MOG antibody disease and AQP-4 positive NMOSD are thought to have distinct immunological mechanisms. Those with MOG antibody disease do not test positive for the NMO antibody called aquaporin 4 (AQP-4). Patients with persistently positive antibodies are at risk for recurrent events. Those with MOG Antibody Disease may previously have been diagnosed with Neuromyelitis Optica Spectrum Disorder (NMOSD), Transverse Myelitis (TM), Acute Disseminated Encephalomyelitis (ADEM), Optic Neuritis (ON), or multiple sclerosis (MS) because of the pattern of inflammation it causes including brain, spinal cord and optic nerve damage. 3 The diagnosis is confirmed when MOG antibodies in the blood are found in patients who have repeated inflammatory attacks of the central nervous system. 1,2 While the function of this glycoprotein is not exactly known, MOG is a target of the immune system in this disease. Myelin oligodendrocyte glycoprotein (MOG) is a protein that is located on the surface of myelin sheaths in the central nervous system. MOG antibody disease (MOGAD) is a recently coined neuro-inflammatory condition that preferentially causes inflammation in the optic nerve but can also cause inflammation in the spinal cord and brain. This information sheet has been reviewed and approved by members of SRNA’s Medical and Scientific Council. COVID-19 and Rare Neuroimmune Disorders.
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