It is thought that myelin constituents such as myelin basic protein, MOG, and myelin proteolipid protein share antigenic determinants with that of an inciting pathogen in a process termed molecular mimicry. ADEM has also been referred to as post-infectious encephalomyelitis based on this suspected mechanism of a pathogen triggering the aberrant immune response. A multidisciplinary approach to the follow-up of a child with ADEM, involving the expertise of neurologists, neuropsychologists, and physiatrists, among other clinicians, is important to optimize disease recovery and facilitate surveillance for potential relapses of acute demyelination.ĪDEM is postulated to be an autoimmune disorder in which an environmental stimulus triggers a dysfunctional, exaggerated immune response in genetically susceptible individuals. While long-term prognosis and physical functioning is good, increasing data suggest that children with ADEM can have persistent neurocognitive deficits. Early initiation of physical, occupational, and speech therapy, when applicable, can help facilitate earlier and more complete recovery. Once testing is sufficiently suggestive of an acute demyelinating syndrome, it is critical that treatments directed at reducing inflammation and immune activation are started to decrease the duration and severity of the illness. It is also important to consider whether ADEM represents the first attack of a relapsing inflammatory demyelinating syndrome, particularly myelin oligodendrocyte glycoprotein (MOG) antibody disease or, less likely, multiple sclerosis or neuromyelitis optica spectrum disorder (NMOSD). Diagnostic testing should be aimed at differentiating ADEM from potential infectious, toxic/metabolic, genetic, vascular, and neoplastic mimics. This condition more commonly affects children and is often a monophasic illness with good functional recovery. This review summarizes the current knowledge of the pathogenesis, epidemiology, clinical features, diagnostic evaluation, treatment approaches, and outcomes in pediatric ADEM.Īcute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system (CNS) that typically presents with encephalopathy and multifocal brain lesions. Further research related to the optimal management of pediatric ADEM and its impact on prognosis is needed. Long-term outcomes for ADEM are generally favorable, but some children have significant morbidity related to the severity of acute illness and/or manifest ongoing neurocognitive sequelae. Acute treatment modalities include high-dose intravenous corticosteroids, therapeutic plasma exchange, and intravenous immunoglobulin. Diagnostic evaluation for ADEM involves neuroimaging and laboratory studies to exclude potential infectious, inflammatory, neoplastic, and genetic mimics of ADEM. With increasing awareness, understanding, and testing for myelin oligodendrocyte glycoprotein antibodies, this disease is now known to be a cause of pediatric ADEM and also has the potential to be relapsing. ADEM is often a monophasic disorder, in contrast to other demyelinating disorders such as multiple sclerosis and neuromyelitis optica spectrum disorder. Although ADEM is thought to be a post-infectious disorder, the etiology is still poorly understood. Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system that typically presents in childhood and is associated with encephalopathy and multifocal brain lesions.
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