eprintid: 10073403 rev_number: 22 eprint_status: archive userid: 608 dir: disk0/10/07/34/03 datestamp: 2019-05-08 16:33:24 lastmod: 2021-09-22 22:15:07 status_changed: 2019-05-08 16:33:24 type: article metadata_visibility: show creators_name: Wong, YYM creators_name: Hacohen, Y creators_name: Armangue, T creators_name: Wassmer, E creators_name: Verhelst, H creators_name: Hemingway, C creators_name: van Pelt, ED creators_name: Catsman-Berrevoets, CE creators_name: Hintzen, RQ creators_name: Deiva, K creators_name: Lim, MJ creators_name: Rostasy, K creators_name: Neuteboom, RF title: Paediatric acute disseminated encephalomyelitis followed by optic neuritis: disease course, treatment response and outcome ispublished: pub subjects: GOSH divisions: UCL divisions: B02 divisions: C07 divisions: D07 divisions: F87 keywords: acute disseminated encephalomyelitis, anti‐myelin oligodendrocyte glycoprotein antibodies, optic neuritis, paediatric treatment response note: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. abstract: Background and purpose: Acute disseminated encephalomyelitis followed by optic neuritis (ADEM‐ON) is a rare demyelinating syndrome that is different from multiple sclerosis and neuromyelitis optica spectrum disorder. The aim of this study was to describe the disease course, treatment response and outcome of children with ADEM‐ON. / Methods: Children of <18 years of age were identified from six countries of the EU Paediatric Demyelinating Disease Consortium. Patients fulfilled the diagnostic criteria for ADEM followed by at least one ON. Anti‐myelin oligodendrocyte glycoprotein (MOG) antibodies were tested in all patients. / Results: In this study of 17 patients (nine boys) with ADEM‐ON, anti‐myelin oligodendrocyte glycoprotein (MOG) antibodies were identified in 16 patients. Age at onset was 6.1 years (interquartile range, 5.1–9.2 years). Twelve patients received oral prednisolone and 10 received maintenance immunosuppression (e.g. azathioprine, intravenous immunoglobulins, Rituximab). During a follow‐up of 5.3 years (interquartile range, 1.8–10.2 years), 54 relapses occurred with a median of 3 relapses per patient (range, 1–9 per patient). Patients relapsed on all treatments but no relapses occurred on a prednisolone dose >10 mg/day. Visual and cognitive residual deficits were common in this group. / Conclusions: Acute disseminated encephalomyelitis followed by optic neuritis is an anti‐MOG antibody‐associated relapsing disorder that can have a heterogeneous disease course. Patients were refractory for maintenance immunosuppression and appeared to be corticosteroid‐dependent. Further international collaborations are now required to unify guidelines in this difficult‐to‐manage group of patients. date: 2018-05 date_type: published publisher: WILEY official_url: https://doi.org/10.1111/ene.13602 oa_status: green full_text_type: other language: eng primo: open primo_central: open_green verified: verified_manual elements_id: 1554063 doi: 10.1111/ene.13602 lyricists_name: Hacohen, Yael lyricists_id: YHACO24 actors_name: Allington-Smith, Dominic actors_id: DAALL44 actors_role: owner full_text_status: public publication: European Journal of Neurology volume: 25 number: 5 pagerange: 782-786 pages: 5 issn: 1468-1331 citation: Wong, YYM; Hacohen, Y; Armangue, T; Wassmer, E; Verhelst, H; Hemingway, C; van Pelt, ED; ... Neuteboom, RF; + view all <#> Wong, YYM; Hacohen, Y; Armangue, T; Wassmer, E; Verhelst, H; Hemingway, C; van Pelt, ED; Catsman-Berrevoets, CE; Hintzen, RQ; Deiva, K; Lim, MJ; Rostasy, K; Neuteboom, RF; - view fewer <#> (2018) Paediatric acute disseminated encephalomyelitis followed by optic neuritis: disease course, treatment response and outcome. European Journal of Neurology , 25 (5) pp. 782-786. 10.1111/ene.13602 <https://doi.org/10.1111/ene.13602>. Green open access document_url: https://discovery.ucl.ac.uk/id/eprint/10073403/3/Hacohen_EJN%20Wong%20et%20al_ADEM-ON%20-%20disease%20course%20and%20treatment_v2.doc%20yh%20030118.pdf