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JAMA Neurol. 2018 Apr 1;75(4):478-487. doi: 10.1001/jamaneurol.2017.4601.

Disease Course and Treatment Responses in Children With Relapsing Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease.

Author information

Department of Neuroinflammation, Queen Square Multiple Sclerosis Centre, University College London Institute of Neurology, London, United Kingdom.
Department of Paediatric Neurology, Great Ormond Street Hospital for Children, London, United Kingdom.
Department of Neurology, Erasmus University Medical Center, Rotterdam, the Netherlands.
Division of Paediatric Neurology, Department of Paediatrics, Medical University of Innsbruck, Innsbruck, Austria.
Neurology Department, John Radcliffe Hospital, Oxford, United Kingdom.
Department of Paediatric Neurology, Birmingham Children's Hospital, Birmingham, United Kingdom.
Division of Pediatric Neurology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Centre Hospitalier, Universitaire Vaudois of Lausanne, Lausanne, Switzerland.
Department of Pediatric Neurology, Groupement Hospitalier Est, Hospices Civils de Lyon, Lyon, France.
National Referral Center for Neuro-Inflammatory Diseases and Pediatric Neurology Department, Assistance Publique-Hôpitaux de Paris, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
Service de Neuropédiatrie, University Paris Sud, Le Kremlin-Bicêtre, France.
Pediatric Neurology Unit, Schneider Children's Medical Center, Tel-Aviv, Israel.
Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
Institute of Neuroscience, Newcastle University, Newcastle, United Kingdom.
Department of Pediatrics, Olga Hospital, Stuttgart, Germany.
Service de Neurologie A and Eugène Devic Foundation Against Multiple Sclerosis, Observatoire Français de la Sclérose en Plaques, Hôpital Neurologique Pierre Wertheimer-Groupement Hospitalier Est, Hospices Civils de Lyon, Bron, France.
Lyon's Neuroscience Research Center, INSERM U 1028/Centre National de la Recherche Scientifique 5292, Université de Lyon, Université Claude Bernard Lyon 1, Lyon, France.
Department of Pediatric Neurology, Children's Hospital Datteln, University Witten/Herdecke, Witten, Germany.
Children's Neurosciences, Evelina London Children's Hospital at Guy's and St Thomas' National Health Service Foundation Trust, King's Health Partners Academic Health Science Centre, London, United Kingdom.
Faculty of Life Sciences and Medicine, King's College London, London, United Kingdom.



Myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) are consistently identified in a range of demyelinating disorders in adults and children. Current therapeutic strategies are largely center specific, and no treatments have been formally evaluated.


To examine the clinical phenotypes, treatment responses, and outcomes of children with relapsing MOG-Ab-associated disease.

Design, Setting, and Participants:

This study prospectively collected demographic, clinical, and radiologic data from 102 patients from 8 countries of the EU Paediatric Demyelinating Disease Consortium from January 1, 2014, through December 31, 2016. Patients were treated according to local protocols.

Main Outcomes and Measures:

Annualized relapse rates (ARRs) and Expanded Disability Status Scale (EDSS) scores before and during treatment with disease-modifying drugs (DMDs).


A total of 102 children were identified (median [range] age, 7.0 [1.5-7.9] years; male to female ratio, 1.0:1.8; white to other race/ethnicity ratio, 3.6:1.0). Original diagnoses were neuromyelitis optica spectrum disorder (44 patients [43.1%]), acute disseminated encephalomyelitis followed by optic neuritis (20 [19.6%]), multiphasic disseminated encephalomyelitis (20 [19.6%]), and relapsing optic neuritis (18 [17.6%]). In all, 464 demyelinating events were reported. Treated patients had more relapses (median, 3.0; range, 1.0-17.0) than untreated patients (median, 1.0; range 1.0-7.0) (P = .009) and higher EDSS scores (median, 1.5; interquartile range, 0-2.5) than untreated patients (median, 1.0; interquartile range, 0-1.5) (P < .001). Fifty-two children (51.0%) received DMDs: 28 (53.8%) were treated with 1 DMD, 17 (32.7%) with 2, and 7 (13.5%) with 3 or more sequential DMDs. Patients relapsed during all treatments, with a total of 127 relapses on treatment reported. No changes in median ARR and EDSS score were observed between the preinitiation and postinitiation phases of interferon beta and glatiramer acetate treatment (n = 11). The median ARR was reduced from 1.84 to 1.0 with azathioprine (n = 20, P < .001), 1.79 to 0.52 with mycophenolate mofetil (n = 15, P = .003), and 2.12 to 0.67 with rituximab (n = 9, P < .001), although the median EDSS score remained unchanged. An improvement in ARR (from 2.16 to 0.51, P < .001) and EDSS score (from 2.2 to 1.2, P = .01) was observed in the 12 patients treated with regular intravenous immunoglobulins.

Conclusions and Relevance:

Although commonly used to treat patients with multiple sclerosis, DMDs were not associated with clinical improvement in children with MOG-Ab-associated disease, whereas azathioprine, mycophenolate mofetil, rituximab, and particularly intravenous immunoglobulins were associated with a reduction in relapse frequency. A correct diagnosis of relapsing MOG-Ab-associated disorders is therefore important to optimize immune treatment.

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