dc.contributor.author | Ramanathan, Sudarshini | |
dc.contributor.author | Mohammad, Shekeeb | |
dc.contributor.author | Tantsis, Esther | |
dc.contributor.author | Nguyen, Tina Kim | |
dc.contributor.author | Merheb, Vera | |
dc.contributor.author | Fung, Victor SC | |
dc.contributor.author | White, Owen Bruce | |
dc.contributor.author | Broadley, Simon | |
dc.contributor.author | Lechner-Scott, Jeannette | |
dc.contributor.author | Vucic, Steve | |
dc.contributor.author | Henderson, Andrew PD | |
dc.contributor.author | Barnett, Michael Harry | |
dc.contributor.author | Reddel, Stephen W | |
dc.contributor.author | Brilot, Fabienne | |
dc.contributor.author | Dale, Russell C | |
dc.date.accessioned | 2019-07-05T12:32:43Z | |
dc.date.available | 2019-07-05T12:32:43Z | |
dc.date.issued | 2018 | |
dc.identifier.issn | 0022-3050 | |
dc.identifier.doi | 10.1136/jnnp-2017-316880 | |
dc.identifier.uri | http://hdl.handle.net/10072/377228 | |
dc.description.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. | |
dc.description.peerreviewed | Yes | |
dc.language | English | |
dc.language.iso | eng | |
dc.publisher | BMJ Group | |
dc.publisher.place | United Kingdom | |
dc.relation.ispartofpagefrom | 127 | |
dc.relation.ispartofpageto | 137 | |
dc.relation.ispartofissue | 2 | |
dc.relation.ispartofjournal | Journal of Neurology, Neurosurgery and Psychiatry | |
dc.relation.ispartofvolume | 89 | |
dc.subject.fieldofresearch | Biomedical and clinical sciences | |
dc.subject.fieldofresearch | Psychology | |
dc.subject.fieldofresearchcode | 32 | |
dc.subject.fieldofresearchcode | 52 | |
dc.title | Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination | |
dc.type | Journal article | |
dc.type.description | C1 - Articles | |
dc.type.code | C - Journal Articles | |
dcterms.license | http://creativecommons.org/licenses/by/4.0/ | |
dc.description.version | Version of Record (VoR) | |
gro.rights.copyright | © The Author(s) 2018. 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. | |
gro.hasfulltext | Full Text | |
gro.griffith.author | Broadley, Simon | |