Abstract
Myelin oligodendrocyte glycoprotein (MOG) autoantibodies have gained increasing attention in recent years as an important biomarker for central nervous system demyelinating disorders. MOG antibodies have been identified in a wide spectrum of demyelinating syndromes, with a particular predominance in pediatric patients. The clinical spectrum of MOG antibody-associated disease (MOGAD) continues to expand and differs substantially between children and adults. The characteristic clinical manifestations of MOGAD include acute disseminated encephalomyelitis (ADEM) in young children, whereas optic neuritis (ON) and transverse myelitis (TM) are more commonly observed in older children. A proportion of patients experience a relapsing disease course, presenting with ADEM followed by one or more episodes of optic neuritis (ADEM-ON), multiphasic disseminated encephalomyelitis (MDEM), recurrent optic neuritis (RON), or a relapsing phenotype resembling neuromyelitis optica spectrum disorder (NMOSD). More recently, the disease spectrum has been further expanded to include distinctive clinical and radiological phenotypes, such as encephalitis-like presentations, leukodystrophy-like phenotypes, and other unclassifiable manifestations. This review summarizes the current expert consensus recommendations regarding serum MOG antibody testing in pediatric patients, as well as the diagnostic approach and management of MOG antibody-associated disease. In addition, it presents the pediatric clinical classification of MOGAD, including phenotype definitions and their key characteristics, as proposed by the European Pediatric MOG Consortium Consensus Group. This classification is expected to improve clinical practice and provide an essential framework for future research on pediatric MOG antibody-associated disease.