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Assessment and Management of Acute Disseminated Encephalomyelitis (ADEM) in the Pediatric Patient

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. Although ADEM is thought to be a post-infectious disorder, the etiology is still...

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Autor principal: Wang, Cynthia X.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer International Publishing 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8026386/
https://www.ncbi.nlm.nih.gov/pubmed/33830467
http://dx.doi.org/10.1007/s40272-021-00441-7
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author Wang, Cynthia X.
author_facet Wang, Cynthia X.
author_sort Wang, Cynthia X.
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description 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. Although ADEM is thought to be a post-infectious disorder, the etiology is still poorly understood. ADEM is often a monophasic disorder, in contrast to other demyelinating disorders such as multiple sclerosis and neuromyelitis optica spectrum disorder. 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. Diagnostic evaluation for ADEM involves neuroimaging and laboratory studies to exclude potential infectious, inflammatory, neoplastic, and genetic mimics of ADEM. Acute treatment modalities include high-dose intravenous corticosteroids, therapeutic plasma exchange, and intravenous immunoglobulin. 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. Further research related to the optimal management of pediatric ADEM and its impact on prognosis is needed. This review summarizes the current knowledge of the pathogenesis, epidemiology, clinical features, diagnostic evaluation, treatment approaches, and outcomes in pediatric ADEM.
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spelling pubmed-80263862021-04-08 Assessment and Management of Acute Disseminated Encephalomyelitis (ADEM) in the Pediatric Patient Wang, Cynthia X. Paediatr Drugs Therapy in Practice 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. Although ADEM is thought to be a post-infectious disorder, the etiology is still poorly understood. ADEM is often a monophasic disorder, in contrast to other demyelinating disorders such as multiple sclerosis and neuromyelitis optica spectrum disorder. 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. Diagnostic evaluation for ADEM involves neuroimaging and laboratory studies to exclude potential infectious, inflammatory, neoplastic, and genetic mimics of ADEM. Acute treatment modalities include high-dose intravenous corticosteroids, therapeutic plasma exchange, and intravenous immunoglobulin. 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. Further research related to the optimal management of pediatric ADEM and its impact on prognosis is needed. This review summarizes the current knowledge of the pathogenesis, epidemiology, clinical features, diagnostic evaluation, treatment approaches, and outcomes in pediatric ADEM. Springer International Publishing 2021-04-08 2021 /pmc/articles/PMC8026386/ /pubmed/33830467 http://dx.doi.org/10.1007/s40272-021-00441-7 Text en © This is a U.S. government work and not under copyright protection in the U.S.; foreign copyright protection may apply 2021 This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic.
spellingShingle Therapy in Practice
Wang, Cynthia X.
Assessment and Management of Acute Disseminated Encephalomyelitis (ADEM) in the Pediatric Patient
title Assessment and Management of Acute Disseminated Encephalomyelitis (ADEM) in the Pediatric Patient
title_full Assessment and Management of Acute Disseminated Encephalomyelitis (ADEM) in the Pediatric Patient
title_fullStr Assessment and Management of Acute Disseminated Encephalomyelitis (ADEM) in the Pediatric Patient
title_full_unstemmed Assessment and Management of Acute Disseminated Encephalomyelitis (ADEM) in the Pediatric Patient
title_short Assessment and Management of Acute Disseminated Encephalomyelitis (ADEM) in the Pediatric Patient
title_sort assessment and management of acute disseminated encephalomyelitis (adem) in the pediatric patient
topic Therapy in Practice
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8026386/
https://www.ncbi.nlm.nih.gov/pubmed/33830467
http://dx.doi.org/10.1007/s40272-021-00441-7
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