• Sudarshini Ramanathan, Shekeeb Mohammad, Esther Tantsis, Tina Kim Nguyen, Vera Merheb, FungVictor S CVSCDepartment of Neurology, Westmead Hospital, Westmead, New South Wales, Australia.University of Sydney, Sydney, New South Wales, Australia., Owen Bruce White, Simon Broadley, Jeannette Lechner-Scott, Steve Vucic, HendersonAndrew P DAPDDepartment of Neurology, Westmead Hospital, Westmead, New South Wales, Australia.University of Sydney, Sydney, New South Wales, Australia.Department of Ophthalmology, Westmead Hospital, Sydney, New South Wales, Australia., Michael Harry Barnett, Stephen W Reddel, Fabienne Brilot, Russell C Dale, and Australasian and New Zealand MOG Study Group.
• Brain Autoimmunity Group, Institute for Neuroscience and Muscle Research, The Kids Research Institute at the Children's Hospital, Westmead, New South Wales, Australia.
• J. Neurol. Neurosurg. Psychiatr. 2018 Feb 1; 89 (2): 127-137.

ObjectiveWe characterised the clinical course, treatment and outcomes in 59 patients with relapsing myelin oligodendrocyte glycoprotein (MOG) antibody-associated demyelination.MethodsWe evaluated clinical phenotypes, annualised relapse rates (ARR) prior and on immunotherapy and Expanded Disability Status Scale (EDSS), in 218 demyelinating episodes from 33 paediatric and 26 adult patients.ResultsThe most common initial presentation in the cohort was optic neuritis (ON) in 54% (bilateral (BON) 32%, unilateral (UON) 22%), followed by acute disseminated encephalomyelitis (ADEM) (20%), which occurred exclusively in children. ON was the dominant phenotype (UON 35%, BON 19%) of all clinical episodes. 109/226 (48%) MRIs had no brain lesions. Patients were steroid responsive, but 70% of episodes treated with oral prednisone relapsed, particularly at doses <10 mg daily or within 2 months of cessation. Immunotherapy, including maintenance prednisone (P=0.0004), intravenous immunoglobulin, rituximab and mycophenolate, all reduced median ARRs on-treatment. Treatment failure rates were lower in patients on maintenance steroids (5%) compared with non-steroidal maintenance immunotherapy (38%) (P=0.016). 58% of patients experienced residual disability (average follow-up 61 months, visual loss in 24%). Patients with ON were less likely to have sustained disability defined by a final EDSS of ≥2 (OR 0.15, P=0.032), while those who had any myelitis were more likely to have sustained residual deficits (OR 3.56, P=0.077).ConclusionRelapsing MOG antibody-associated demyelination is strongly associated with ON across all age groups and ADEM in children. Patients are highly responsive to steroids, but vulnerable to relapse on steroid reduction and cessation.© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

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