Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination. Issue 2 (15th November 2017)

• Record Type: Journal Article
• Authors: Ramanathan, Sudarshini; Mohammad, Shekeeb; Tantsis, Esther; Nguyen, Tina Kim; Merheb, Vera; Fung, Victor S C; White, Owen Bruce; Broadley, Simon; Lechner-Scott, Jeannette; Vucic, Steve; Henderson, Andrew P D; Barnett, Michael Harry; Reddel, Stephen W; Brilot, Fabienne; Dale, Russell C
• Other Names: author non-byline.; Andrews Pi author non-byline.; Barton Jl author non-byline.; Burrow Jnc author non-byline.; Butzkueven H author non-byline.; Cairns Ag author non-byline.; Calvert S author non-byline.; Caruana P author non-byline.; Chelakkadan S author non-byline.; Clark D author non-byline.; Fraser Cl author non-byline.; Freeman Jl author non-byline.; Gill D author non-byline.; Grattan-smith Pj author non-byline.; Gupta S author non-byline.; Hardy Ta author non-byline.; Kothur K author non-byline.; Ling Sr author non-byline.; Lopez Ja author non-byline.; Malone S author non-byline.; Marriott Mp author non-byline.; Nosadini M author non-byline.; O'grady Gl author non-byline.; Orr Cf author non-byline.; Ouvrier R author non-byline.; Parratt J author non-byline.; Patrick E author non-byline.; Pilli D author non-byline.; Riminton Ds author non-byline.; Riney K author non-byline.; Rodriguez-casero V author non-byline.; Ryan Mm author non-byline.; Scheffer Ie author non-byline.; Shah Uh author non-byline.; Shuey N author non-byline.; Spooner Cg author non-byline.; Subramanian Gm author non-byline.; Tea F author non-byline.; Thomas T author non-byline.; Thompson J author non-byline.; Troedson C author non-byline.; Ware Tl author non-byline.; Webster Ri author non-byline.; Yiannikas C author non-byline.; Yiu Em author non-byline.; Zou A author non-byline.;
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 89:Issue 2(2018)
• Page Start: 127
• Page End: 137
• Publication Date: 2017-11-15
• Subjects: myelin oligodendrocyte glycoprotein antibodies -- optic neuritis -- acute disseminated encephalomyelitis -- therapy -- outcomes; Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp-2017-316880
• Languages: English
• ISSNs: 0022-3050

Abstract

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.