Abstract
Imaging plays a crucial role in differentiating the spectrum of paediatric acquired demyelinating syndromes (ADS), which apart from myelin oligodendrocyte glycoprotein antibody associated disorders (MOGAD) includes paediatric multiple sclerosis (MS), aquaporin-4 antibody neuromyelitis optica spectrum disorders (NMOSD) and unclassified patients with both monophasic and relapsing ADS. In contrast to the imaging characteristics of children with MS, children with MOGAD present with diverse imaging patterns which correlate with the main demyelinating phenotypes as well as age at presentation. In this review we describe the common neuroradiological features of children with MOGAD such as acute disseminated encephalomyelitis, optic neuritis, transverse myelitis, AQP4 negative NMOSD. In addition, we report newly recognized presentations also associated with MOG-ab such as the 'leukodystophy-like' phenotype and autoimmune encephalitis with predominant involvement of cortical and deep grey matter structures. We further delineate the features, which may help to distinguish MOGAD from other ADS and discuss the future role of MR-imaging in regards to treatment decisions and prognosis in children with MOGAD. Finally, we propose an MRI protocol for routine examination and discuss new imaging techniques, which may help to better understand the neurobiology of MOGAD.
Highlights
Myelin oligodendrocyte glycoprotein antibodies (MOG-abs) can be detected in children presenting with a range of paediatric acquired demyelinating syndromes (ADS) such as acute disseminated encephalomyelitis (ADEM), aquaporin-4 (AQP4) antibody negative neuromyelitis optica spectrum disorders (NMOSD), ADEM followed by optic neuritis (ADEMON), multiphasic disseminated encephalomyelitis (MDEM), and optic neuritis (ON)
Until recently MOG-ab-associated disorders (MOGAD) were thought to have a more benign outcome compared to other recurrent demyelinating diseases including multiple sclerosis (MS) or AQP4-ab-positive NMOSD
Imaging plays a crucial role in differentiating the spectrum of ADS, which apart from MOGAD includes paediatric MS, AQP4-ab-positive NMOSD and other less well-defined subgroups
Summary
Myelin oligodendrocyte glycoprotein antibodies (MOG-abs) can be detected in children presenting with a range of paediatric acquired demyelinating syndromes (ADS) such as acute disseminated encephalomyelitis (ADEM), aquaporin-4 (AQP4) antibody negative neuromyelitis optica spectrum disorders (NMOSD), ADEM followed by optic neuritis (ADEMON), multiphasic disseminated encephalomyelitis (MDEM), and optic neuritis (ON). MOGAD and AQP4-ab-positive disease display different imaging characteristics MS, but overlap in brain lesion distribution despite targeting distinct cell types (oligodendrocytes vs astrocytes) [20]. Curvilinear lesions involving subcortical U-fibres seemed to be a distinguishing feature between MS, AQP4-ab-positive NMOSD, and MOGAD in a cohort of mostly adult patients, but curvilinear lesions appear in young children with MOG-ab-positive ADEM (Figure 1E) [11, 24]. Recognized presentations More recently described phenotypes of MOGAD with characteristic clinical and radiological features include a leukodystrophy-like phenotype with confluent white matter lesions and autoimmune encephalitis (AE) with MOG-abs and predominant involvement of cortical and deep gray matter structures [13, 18, 21, 29]. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) with detection of MOG-abs later in the course has so far only been described in one adult patient [35]
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