E.U. paediatric MOG consortium consensus: Part 5 – Treatment of paediatric myelin oligodendrocyte glycoprotein antibody-associated disorders

Bruijstens, Arlette L.; Wendel, Eva-Maria; Lechner, Christian; Bartels, Frank; Finke, Carsten; Breu, Markus; Flet‐Berliac, Lorraine; Chalus, Aliénor de; Adamsbaum, C.; Capobianco, Marco; Giorgi, Laetitia; Hacohen, Yael; Hemingway, Cheryl; Wassmer, Evangeline; Lim, Ming; Baumann, Matthias; Wickström, Ronny; Armangué, Thaís; Rostásy, Kevin; Deiva, Kumaran; Neuteboom, Rinze F.

doi:10.1016/j.ejpn.2020.10.005

Cited by 76 publications

(66 citation statements)

References 81 publications

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“…Recently, E.U. pediatric MOG consortium consensus proposed a recommendation for steroid tapering with oral prednisone after acute treatment for <3 months to avoid side effects (38). A study reported by Waters et al (37) also considered that commencing long-term immunomodulatory therapy for patients immediately following their first episodes was not appropriate because 72% of persistently seropositive patients in their study remained monophasic at the last follow-up.…”

Section: Discussionmentioning

confidence: 99%

Clinical and Prognostic Analysis of Autoantibody-Associated CNS Demyelinating Disorders in Children in Southwest China

Sun

Fan

et al. 2021

Front. Neurol.

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Objective: To analyze the positive and recurrence rates of different autoantibody-associated demyelination disorders in children in Southwest China, and describe the clinical, radiological, and prognostic features of the myelin oligodendrocyte glycoprotein antibody (MOG-ab) and aquaporin-4 antibody (AQP4-ab) associated disease. This study also summarizes steroid maintenance therapy approaches for MOG-ab-positive children.Methods: A total of 160 children presenting with acquired demyelinating syndromes (ADS) between January 2016 and December 2019 were tested for MOG-ab and AQP4-ab. Clinical data, MRI scans, and survival analyses were compared between MOG-ab-positive and AQP4-ab-positive children. Evolution of serologic status and treatment response to immunosuppressants were collected in MOG-ab-positive children.Results: Of the 160 included children, the MOG-ab positivity rate (47.4%) was significantly higher than the AQP4-ab (5%) positivity rate. The recurrence rate for AQP4-ab disease (71.4%) was higher than that of MOG-ab disease (30.1%). For 135 children with both MOG-ab and AQP4-ab tested, the median age at onset was 7 (interquartile range [IQR] 5–10) years, and the median follow-up period was 19 (IQR 13–27.5) months. MOG-ab-positive children more frequently presented with acute disseminated encephalomyelitis, had deep gray matter lesions on MRI, had a better clinical and radiological recovery, and were less likely to have sustained disability than AQP4-ab-positive children. In MOG-ab-positive and AQP4-ab-positive children, maintenance therapy was a protective factor for recurrence, but presenting optic neuritis was a predictor of earlier relapse. A high Expanded Disability Status Scale score at onset was associated with sustained disability. Steroid maintenance therapy longer than 6 months after the initial attack was associated with a lower risk of a second relapse in MOG-ab-positive children. On serial serum MOG antibody analysis, clinical relapse occurred in 34.6% of children with persistent seropositivity, but none of the children who converted to seronegative status experienced relapse.Conclusion: The MOG antibody is more common in children with ADS than the AQP4 antibody. MOG-ab-positive children are characterized by distinct clinical and radiological features. Although some MOG-ab-positive children experience relapsing courses or have persistently seropositive status, they still predict a better outcome than AQP4-ab-positive children.

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Section: Discussionmentioning

confidence: 99%

Clinical and Prognostic Analysis of Autoantibody-Associated CNS Demyelinating Disorders in Children in Southwest China

Sun

Fan

et al. 2021

Front. Neurol.

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“…Importantly, clinical symptoms and radiologic features can fluctuate during the acute phase of ADEM (up to three months) [2], mainly during weaning off immunomodulatory treatment. Therefore, during this acute ADEM period it is important to be aware of the possibility of a "flare-up", which does not reflect a true relapse [125]. A relapse was defined by the consensus group as a new clinical episode accompanied by radiological evidence depending on the subtype of MOGAD, appearing at least one month subsequently to the last acute attack.…”

Section: Multiphasic Disseminated Encephalomyelitis (Mdem)mentioning

confidence: 99%

“…A subgroup of children with initial ADEM presentation continue to have demyelinating episode(s) limited to the optic nerve (ADEM-ON) [83]. As for MDEM, during the acute phase of ADEM (up to three months) the possibility of a "flare-up", instead of a true relapse, should be considered [125], as optic nerve involvement can also occur during the acute phase of ADEM [2,31].…”

Section: Adem-onmentioning

confidence: 99%

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

consortium¹,

Bruijstens

Wendel³

et al. 2020

European Journal of Paediatric Neurology

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108

114

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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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“…Four working groups were formed on the different aspects of MOGAD in children such as clinical characteristics of the various subtypes, radiological features, role of MOG-ab in the disease process and outcome of children with MOGAD [1e4]. The whole group then worked on a statement regarding the acute and maintenance treatment of paediatric MOGAD [5].…”

mentioning

confidence: 99%

“…Again, due to the rarity of MOGAD and in particular the relapsing forms and lack of structured prospective treatment evaluations the consortium members formulated two protocols e one addressing the acute management and the other maintenance therapy -that respected different treatment strategies within the group. In addition, part 5 also provides rationales for both initiation and cessation of treatment options [5].…”

mentioning

confidence: 99%

Editorial on EJPN focus section on E.U. paediatric MOG consortium consensus statements

Neuteboom

Deiva

Rostásy

2020

European Journal of Paediatric Neurology

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E.U. paediatric MOG consortium consensus: Part 5 – Treatment of paediatric myelin oligodendrocyte glycoprotein antibody-associated disorders

Cited by 76 publications

References 81 publications

Clinical and Prognostic Analysis of Autoantibody-Associated CNS Demyelinating Disorders in Children in Southwest China

Clinical and Prognostic Analysis of Autoantibody-Associated CNS Demyelinating Disorders in Children in Southwest China

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

Editorial on EJPN focus section on E.U. paediatric MOG consortium consensus statements

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