The DOI matches its Crossref record; 42 of the 42 references it cites were found in a registry or an index.
Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination
Open accessAbstract
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.
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Cites
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Myelin-oligodendrocyte glycoprotein antibody-associated disease
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Metadata
- Authors
- Ramanathan, S., Mohammad, S., Tantsis, E., Nguyen, T. K., Merheb, V., Fung, V. S. C., White, O. B., Broadley, S., Lechner-Scott, J., Vucic, S., Henderson, A. P. D., Barnett, M. H., Reddel, S. W., Brilot, F., & Dale, R. C.
- Venue
- Journal of Neurology, Neurosurgery & Psychiatry
- Publisher
- BMJ
- Published
- 2017-11-15
- Volume
- 89
- Issue
- 2
- Pages
- 127-137
- ISSN
- 0022-3050 (ISSN-L)
- 1468-330X
- OpenAlex
- W2770558705
- Cited by
- 572
- References
- 42
- FWCI
- 34.68
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- Ramanathan, S., Mohammad, S., Tantsis, E., Nguyen, T. K., Merheb, V., Fung, V. S. C., White, O. B., Broadley, S., Lechner-Scott, J., Vucic, S., Henderson, A. P. D., Barnett, M. H., Reddel, S. W., Brilot, F., & Dale, R. C. (2018). Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination. Journal of Neurology, Neurosurgery & Psychiatry. https://doi.org/10.1136/jnnp-2017-316880
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