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Review
. 2020 Nov 3;7(11):210.
doi: 10.3390/children7110210.

Acute Disseminated Encephalomyelitis: Current Perspectives

Renata Barbosa Paolilo  1 , Kumaran Deiva  2   3   4 , Rinze Neuteboom  5 , Kevin Rostásy  6 , Ming Lim  7   8   9
Affiliations
Review

Acute Disseminated Encephalomyelitis: Current Perspectives

Renata Barbosa Paolilo et al. Children (Basel). .

Abstract

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated central nervous system (CNS) disorder, characterized by polyfocal symptoms, encephalopathy and typical magnetic resonance imaging (MRI) findings, that especially affects young children. Advances in understanding CNS neuroimmune disorders as well as the association of myelin oligodendrocyte glycoprotein antibody (MOG-Ab) with both monophasic and recurrent forms of ADEM have led to new insights into its definition, management and outcome. In this review, we aim to provide an update based on current epidemiologic, clinical, radiological and immunopathological aspects and clinical outcome of ADEM.

Keywords: acquired demyelination syndrome (ADS); acute disseminated encephalomyelitis (ADEM); multiple sclerosis (MS); myelin oligodendrocyte glycoprotein antibody (MOG-Ab); neuromyelitis optica spectrum disorder (NMOSD); optic neuritis (ON).

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Spectrum of acquired demyelinating syndromes. ADEM, acute disseminated encephalomyelitis; MOG, myelin oligodendrocyte glycoprotein; AQP4 +, aquaporin-4; M-ADEM, multiphasic-ADEM; ADEM-ON, ADEM followed by at least one optic neuritis; NMOSD, neuromyelitis optica spectrum disorder; MS, multiple sclerosis (adapted from Neuteboom et al. [16]).
Figure 2
Figure 2
Typical magnetic resonance imaging features of ADEM: (A) coronal fluid-attenuated inversion recovery (FLAIR) image showing asymmetrical bilateral subcortical white matter abnormalities; (B) axial T2-weighted image depicting asymmetrical bilateral white and gray matter abnormalities; (C) axial T2-weighted image showing typical cerebellar peduncle lesions.
Figure 3
Figure 3
Children with acute disseminated encephalomyelitis (ADEM) and persistent myelin oligodendrocyte glycoprotein antibody (MOGpos-ADEM) are at risk of developing one of three recurrent demyelinating syndromes in addition to less well-characterized manifestations: multiphasic ADEM (MDEM), neuromyelitis optica spectrum disorder (NMOSD) generally with longitudinally extensive transverse myelitis (LETM) or recurrent optic neuritis (ON).
See this image and copyright information in PMC

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