In the last few years, the MOG-Ab–associated disorder (MOGAD) spectrum has been rapidly broadening, and as more data regarding their clinical, radiological, and laboratory findings have become available, their immunopathogenesis has been elucidated. Depending on the clinical assessment, patients can be clinically diagnosed with NMOSD, acute demyelinating encephalomyelitis (ADEM), multiple sclerosis (MS), or isolated ON or TM. The majority of MOG antibody (MOG-Ab)-seropositive patients have optic neuritis (ON), encephalitis with brain demyelinating lesions, and/or transverse myelitis (TM) the new term “MOG-Ab–associated ON, encephalitis, and myelitis” has been suggested to include these patients with CNS demyelinating syndromes and MOG-Ab positivity. MOG was comprehen sively studied as a potential target structure in CNS demyelinating diseases. Myelin oligodendrocyte glycoprotein (MOG) is exclusively expressed in the CNS on the outer surface of the myelin sheath and oligodendrocyte plasma membrane. However, a subgroup of clinically definite NMOSD patients showed AQP4-seronegative results. In 2004, the specific autoantibodies against aquaporin-4 (AQP4), a plentiful water channel on astrocytic endfeet in the CNS, in patients with neuromyelitis optica spectrum disorder (NMOSD) fortified the diagnosis of and research into demyelinating CNS diseases. Diagnosis is based on a combination of clinical manifestation and radiological and laboratory findings. The most common demyelinating CNS diseases have some chance of misdiagnosis that occurs in up to 10% of patients. Inflammatory or immune-mediated demyelinating central nervous system (CNS) diseases are a heterogeneous group that includes mono- and multiphasic diseases with prognoses ranging from benign to fulminant and a variety of different treatment responses. This review summarizes the data regarding MOG-Ab as an impending biological marker for discriminating between these diverse demyelinating CNS diseases and discusses recent developments, clinical applications, and findings regarding the immunopathogenesis of MOG-Ab-associated disorders. A humoral immune reaction against MOG was recently found in monophasic diseases and recurrent/multiphasic clinical progression, particularly in pediatric patients. Using specific cell-based assays, MOG-Ab is becoming a potential biomarker of inflammatory demyelinating disorders of the CNS. MOG-Ab is associated with a wider clinical phenotype not limited to neuromyelitis optica spectrum disorder, with most patients presenting with optic neuritis, acute disseminated encephalomyelitis (ADEM) or ADEM-like encephalitis with brain demyelinating lesions, and/or myelitis. Antibodies against myelin oligodendrocyte glycoprotein (MOG-Ab) have been found in some cases of these demyelinating diseases, particularly in children. This information is provided by the Siegel Rare Neuroimmune Association.Inflammatory or immune-mediated demyelinating central nervous system (CNS) syndromes include a broad spectrum of clinical phenotype and different overlapping diseases. MOG Antibody Disease and AQP-4 positive NMOSD are thought to have distinct immunological mechanisms.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.4 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. Myelin oligodendrocyte glycoprotein (MOG) is a protein that is located on the surface of myelin sheaths in 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.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.4 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.
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