@article {Ramanathanjnnp-2017-316880, author = {Sudarshini Ramanathan and Shekeeb Mohammad and Esther Tantsis and Tina Kim Nguyen and Vera Merheb and Victor S C Fung and Owen Bruce White and Simon Broadley and Jeannette Lechner-Scott and Steve Vucic and Andrew P D Henderson and Michael Harry Barnett and Stephen W Reddel and Fabienne Brilot and Russell C Dale}, editor = {, and , and Andrews, Pi and Barton, Jl and Burrow, Jnc and Butzkueven, H and Cairns, Ag and Calvert, S and Caruana, P and Chelakkadan, S and Clark, D and Fraser, Cl and Freeman, Jl and Gill, D and Grattan-smith, Pj and Gupta, S and Hardy, Ta and Kothur, K and Ling, Sr and Lopez, Ja and Malone, S and Marriott, Mp and Nosadini, M and O{\textquoteright}grady, Gl and Orr, Cf and Ouvrier, R and Parratt, J and Patrick, E and Pilli, D and Riminton, Ds and Riney, K and Rodriguez-casero, V and Ryan, Mm and Scheffer, Ie and Shah, Uh and Shuey, N and Spooner, Cg and Subramanian, Gm and Tea, F and Thomas, T and Thompson, J and Troedson, C and Ware, Tl and Webster, Ri and Yiannikas, C and Yiu, Em and Zou, A}, title = {Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination}, elocation-id = {jnnp-2017-316880}, year = {2017}, doi = {10.1136/jnnp-2017-316880}, publisher = {BMJ Publishing Group Ltd}, 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.}, issn = {0022-3050}, URL = {https://jnnp.bmj.com/content/early/2017/12/07/jnnp-2017-316880}, eprint = {https://jnnp.bmj.com/content/early/2017/12/07/jnnp-2017-316880.full.pdf}, journal = {Journal of Neurology, Neurosurgery \& Psychiatry} }