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Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination.
J Neurol Neurosurg Psychiatry. 2018 02; 89(2):127-137.JN

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.

Authors+Show Affiliations

Brain Autoimmunity Group, Institute for Neuroscience and Muscle Research, The Kids Research Institute at the Children's Hospital, Westmead, New South Wales, Australia. Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia. Department of Neurology, Westmead Hospital, Westmead, New South Wales, Australia.Brain Autoimmunity Group, Institute for Neuroscience and Muscle Research, The Kids Research Institute at the Children's Hospital, Westmead, New South Wales, Australia. Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia. TY Nelson Department of Neurology and Neurosurgery, Children's Hospital, Westmead, New South Wales, Australia.Brain Autoimmunity Group, Institute for Neuroscience and Muscle Research, The Kids Research Institute at the Children's Hospital, Westmead, New South Wales, Australia. Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia. Department of Clinical Medicine, Macquarie University, Sydney, New South Wales, Australia.Brain Autoimmunity Group, Institute for Neuroscience and Muscle Research, The Kids Research Institute at the Children's Hospital, Westmead, New South Wales, Australia. Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia.Brain Autoimmunity Group, Institute for Neuroscience and Muscle Research, The Kids Research Institute at the Children's Hospital, Westmead, New South Wales, Australia. Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia.Department of Neurology, Westmead Hospital, Westmead, New South Wales, Australia. University of Sydney, Sydney, New South Wales, Australia.Ocular Motor Research Laboratory, University of Melbourne, Melbourne, Victoria, Australia. Department of Neurology, Royal Melbourne Hospital, Parkville, Victoria, Australia.School of Medicine, Griffith University, Gold Coast, Queensland, Australia. Department of Neurology, Gold Coast University Hospital, Gold Coast, Queensland, Australia.Department of Neurology, John Hunter Hospital, Newcastle, New South Wales, Australia. Faculty of Medicine and Public Health, Hunter Medical Research Institute, University of Newcastle, Newcastle, New South Wales, Australia.Department of Neurology, Westmead Hospital, Westmead, New South Wales, Australia. University of Sydney, Sydney, New South Wales, Australia.Department of Neurology, Westmead Hospital, Westmead, New South Wales, Australia. University of Sydney, Sydney, New South Wales, Australia. Department of Ophthalmology, Westmead Hospital, Sydney, New South Wales, Australia.Brain and Mind Centre, University of Sydney, Sydney, New South Wales, Australia.Brain and Mind Centre, University of Sydney, Sydney, New South Wales, Australia.Brain Autoimmunity Group, Institute for Neuroscience and Muscle Research, The Kids Research Institute at the Children's Hospital, Westmead, New South Wales, Australia. Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia. Brain and Mind Centre, University of Sydney, Sydney, New South Wales, Australia.TY Nelson Department of Neurology and Neurosurgery, Children's Hospital, Westmead, New South Wales, Australia. Brain and Mind Centre, University of Sydney, Sydney, New South Wales, Australia.No affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

29142145

Citation

Ramanathan, Sudarshini, et al. "Clinical Course, Therapeutic Responses and Outcomes in Relapsing MOG Antibody-associated Demyelination." Journal of Neurology, Neurosurgery, and Psychiatry, vol. 89, no. 2, 2018, pp. 127-137.
Ramanathan S, Mohammad S, Tantsis E, et al. Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination. J Neurol Neurosurg Psychiatry. 2018;89(2):127-137.
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, and Psychiatry, 89(2), 127-137. https://doi.org/10.1136/jnnp-2017-316880
Ramanathan S, et al. Clinical Course, Therapeutic Responses and Outcomes in Relapsing MOG Antibody-associated Demyelination. J Neurol Neurosurg Psychiatry. 2018;89(2):127-137. PubMed PMID: 29142145.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination. AU - Ramanathan,Sudarshini, AU - Mohammad,Shekeeb, AU - Tantsis,Esther, AU - Nguyen,Tina Kim, AU - Merheb,Vera, AU - Fung,Victor S C, AU - White,Owen Bruce, AU - Broadley,Simon, AU - Lechner-Scott,Jeannette, AU - Vucic,Steve, AU - Henderson,Andrew P D, AU - Barnett,Michael Harry, AU - Reddel,Stephen W, AU - Brilot,Fabienne, AU - Dale,Russell C, AU - ,, Y1 - 2017/11/15/ PY - 2017/07/15/received PY - 2017/10/11/revised PY - 2017/10/22/accepted PY - 2017/11/17/pubmed PY - 2019/7/10/medline PY - 2017/11/17/entrez KW - acute disseminated encephalomyelitis KW - myelin oligodendrocyte glycoprotein antibodies KW - optic neuritis KW - outcomes KW - therapy SP - 127 EP - 137 JF - Journal of neurology, neurosurgery, and psychiatry JO - J Neurol Neurosurg Psychiatry VL - 89 IS - 2 N2 - 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. SN - 1468-330X UR - https://www.unboundmedicine.com/medline/citation/29142145/Clinical_course_therapeutic_responses_and_outcomes_in_relapsing_MOG_antibody_associated_demyelination_ L2 - https://jnnp.bmj.com/lookup/pmidlookup?view=long&amp;pmid=29142145 DB - PRIME DP - Unbound Medicine ER -