- Title
- Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination
- Creator
- Ramanathan, Sudarshini; Mohammad, Shekeeb; Henderson, Andrew P. D.; Barnett, Michael Harry; Reddel, Stephen W.; Brilot, Fabienne; Dale, Russell C.; Tantsis, Esther; Nguyen, Tina Kim; Merheb, Vera; Fung, Victor S. C.; White, Owen Bruce; Broadley, Simon; Lechner-Scott, Jeannette; Vucic, Steve
- Relation
- Journal of Neurology, Neurosurgery and Psychiatry Vol. 89, Issue 2, p. 127-137
- Publisher Link
- http://dx.doi.org/10.1136/jnnp-2017-316880
- Publisher
- British Medical Journal (BMJ)
- Resource Type
- journal article
- Date
- 2018
- Description
- 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.
- Subject
- acute disseminated encephalomyelitis; myelin oligodendrocyte glycoprotein antibodies; optic neuritis; outcomes; therapy
- Identifier
- http://hdl.handle.net/1959.13/1459529
- Identifier
- uon:45703
- Identifier
- ISSN:0022-3050
- Rights
- This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.
- Language
- eng
- Full Text
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