Abstract
Over the past few years, increasing interest in the role of autoantibodies against myelin oligodendrocyte glycoprotein (MOG-abs) as a new candidate biomarker in demyelinating central nervous system diseases has arisen. MOG-abs have now consistently been identified in a variety of demyelinating syndromes, with a predominance in paediatric patients. The clinical spectrum of these MOG-ab-associated disorders (MOGAD) is still expanding and differs between paediatric and adult patients. This first part of the Paediatric European Collaborative Consensus emphasises the diversity in clinical phenotypes associated with MOG-abs in paediatric patients and discusses these associated clinical phenotypes in detail. Typical MOGAD presentations consist of demyelinating syndromes, including acute disseminated encephalomyelitis (ADEM) in younger, and optic neuritis (ON) and/or transverse myelitis (TM) in older children. A proportion of patients experience a relapsing disease course, presenting as ADEM followed by one or multiple episode(s) of ON (ADEM-ON), multiphasic disseminated encephalomyelitis (MDEM), relapsing ON (RON) or relapsing neuromyelitis optica spectrum disorders (NMOSD)-like syndromes. More recently, the disease spectrum has been expanded with clinical and radiological phenotypes including encephalitis-like, leukodystrophy-like, and other non-classifiable presentations. This review concludes with recommendations following expert consensus on serologic testing for MOG-abs in paediatric patients, the presence of which has consequences for long-term monitoring, relapse risk, treatments, and for counselling of patient and families. Furthermore, we propose a clinical classification of paediatric MOGAD with clinical definitions and key features. These are operational and need to be tested, however essential for future paediatric MOGAD studies.
Highlights
E.U. paediatric MOG consortium consensus: Part 1 e Classification of clinical phenotypes of paediatric myelin oligodendrocyte glycoprotein antibody-associated disorders
The majority of MOGAD presentations consist of demyelinating syndromes, including typical phenotypes as Acute disseminated encephalomyelitis (ADEM) in younger, and Optic neuritis (ON) and/or TM in older children
Based on the wide diversity of paediatric MOGAD, and the fact that MOG-abs are common in childhood, we recommend to test all paediatric patients presenting with a demyelinating or encephalitic event with abnormalities on brain and/or spinal magnetic resonance imaging (MRI) (Fig. 3)
Summary
The heterogeneous clinical syndrome ADEM predominantly occurs in early childhood and is characterised by encephalopathy, polyfocal neurological deficits and typical magnetic resonance imaging (MRI) abnormalities, which can fluctuate during the acute phase (up to three months after disease onset) [2,43,44]. Besides the anterior optic nerve inflammation, MRI images of MOG-ON showed high rates of longitudinal involvement of the optic nerve in mixed paediatric and adults cohorts (90%), with relative sparing of the chiasm and optic tracts [41,48,50e53,124] The latter being important since this is different from ON associated with AQP4-abs, where patients more often have chiasmal and optic tract involvement [50]. In a study analysing paediatric NMOSD(-like) patients, none of the AQP4-abpositive patients had concomitant ON and (LE)TM, in contrast to 85.7% of MOG-ab-positive patients [33] This simultaneous phenotype has been observed in adult MOG-ab-positive patients in lower percentages (around 40%) [62,66,67], but still was a discriminative feature from AQP4-ab-positive NMOSD [66]. An area postrema syndrome attributable to inflammation in the area postrema is rare and remains a typical feature of AQP4-ab-positive NMOSD
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