E.U. paediatric MOG consortium consensus: Part 1 - Classification of clinical phenotypes of paediatric myelin oligodendrocyte glycoprotein antibody-associated disorders.

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.

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Déclaration de conflit d'intérêts

Declaration of competing interest Arlette L. Bruijstens and Lorraine Flet-Berliac have no conflict of interest to declare. Christian Lechner has served as a consultant for Roche, but has no conflict of interest with this manuscript. Kumaran Deiva has received speaker/consultant honoraria from Novartis and Biogen, but has no conflict of interest with this manuscript. Rinze F. Neuteboom participates in trials by Sanofi and Novartis and has received honoraria from Novartis and Zogenix. Cheryl Hemingway serves as consultant for MS treatment for Biogen, Novartis and AQP4 treatment for Roche. She is an investigator in trials with Biogen, Roche and Novartis, but has no conflict of interest with this manuscript. Evangeline Wassmer has served as a consultant for Novartis and Biogen, PTC therapeutics, GMP-Orphan and Alexion. She is an investigator in trials with Alexion, Biogen Idec, Sanofi and Novartis. Her MS research projects have been funded by the UK MS Society, Action Medical Research and Birmingham Children's Hospital Research Foundation.