Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination

Sudarshini Ramanathan, Shekeeb Mohammad, Esther Tantsis, Tina Kim Nguyen, Vera Merheb, Victor S.C. Fung, Owen Bruce White, Simon Broadley, Jeannette Lechner-Scott, Steve Vucic, Andrew P.D. Henderson, Michael Harry Barnett, Stephen W. Reddel, Fabienne Brilot, Russell C. Dale, on behalf of the Australasian and New Zealand MOG Study Group

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152 Citations (Scopus)

Abstract

Objective We characterised the clinical course, treatment and outcomes in 59 patients with relapsing myelin oligodendrocyte glycoprotein (MOG) antibody-associated demyelination. Methods We 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. Results The 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). Conclusion Relapsing 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.

Original languageEnglish
Pages (from-to)127-137
Number of pages11
JournalJournal of Neurology, Neurosurgery and Psychiatry
Volume89
Issue number2
DOIs
Publication statusPublished - 1 Feb 2018
Externally publishedYes

Keywords

  • acute disseminated encephalomyelitis
  • myelin oligodendrocyte glycoprotein antibodies
  • optic neuritis
  • outcomes
  • therapy

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