학술논문
Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination
Document Type
Academic Journal
Author
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; Andrews, Pi; Barton, Jl; Burrow, Jnc; Butzkueven, H; Cairns, Ag; Calvert, S; Caruana, P; Chelakkadan, S; Clark, D; Fraser, Cl; Freeman, Jl; Gill, D; Grattan-smith, Pj; Gupta, S; Hardy, Ta; Kothur, K; Ling, Sr; Lopez, Ja; Malone, S; Marriott, Mp; Nosadini, M; O’grady, Gl; Orr, Cf; Ouvrier, R; Parratt, J; Patrick, E; Pilli, D; Riminton, Ds; Riney, K; Rodriguez-casero, V; Ryan, Mm; Scheffer, Ie; Shah, Uh; Shuey, N; Spooner, Cg; Subramanian, Gm; Tea, F; Thomas, T; Thompson, J; Troedson, C; Ware, Tl; Webster, Ri; Yiannikas, C; Yiu, Em; Zou, A
Source
Journal of Neurology, Neurosurgery, and Psychiatry. Feb 01, 2018 89(2):127-137
Subject
Language
English
ISSN
0022-3050
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.