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Abstract
Background We investigated the clinical characteristics and outcomes of myelin oligodendrocyte glycoprotein (MOG) antibody-associated autoimmune encephalitis (MOGAE) in adult patients.
Methods From an institutional cohort, we analysed adult patients with MOGAE followed-up for more than 1 year. Disease severity was assessed using the modified Rankin scale (mRS) and Clinical Assessment Scale in Autoimmune Encephalitis scores. Immunotherapy profiles, outcomes and disease relapses were evaluated along with serial brain MRI data.
Results A total of 40 patients were enrolled and categorised into cortical encephalitis (18 patients), limbic encephalitis (LE, 5 patients) and acute disseminated encephalomyelitis (ADEM, 17 patients). 80.0% of patients achieved good clinical outcomes (mRS 0‒2) and 40.0% relapsed. The LE subtype was associated with an older onset age (p=0.004) and poor clinical outcomes (p=0.014) than the other subtypes but with a low rate of relapse (0.0%). 21/25 (84.0%) relapse attacks were associated with an absence or short (≤6 months) immunotherapy maintenance. On MRI, the development of either diffuse cerebral or medial temporal atrophy within the first 6 month was correlated with poor outcomes. MOG-antibody (MOG-Ab) was copresent with anti-N-methyl-D-aspartate receptor (NMDAR)-antibody in 13 patients, in whom atypical clinical presentation (cortical encephalitis or ADEM, p<0.001) and disease relapse (46.2% vs 0.0%, p<0.001) were more frequent compared with conventional NMDAR encephalitis without MOG-Ab.
Conclusions Outcomes are different according to the three phenotypes in MOGAE. Short immunotherapy maintenance is associated with relapse, and brain atrophy was associated with poor outcomes. Patients with dual antibodies of NMDAR and MOG have a high relapse rate.
- AUTOIMMUNE ENCEPHALITIS
Data availability statement
Data are available on reasonable request. The datasets generated during and/or analysed during the current study are available from the corresponding author on request.
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Data availability statement
Data are available on reasonable request. The datasets generated during and/or analysed during the current study are available from the corresponding author on request.
Footnotes
Contributors W-JL acquired study data, performed data analysis and interpretation, and drafted the manuscript. YNK and BK. contributed in the acquisition and management of the laboratory data. JM and K-IP significantly participated in the data acquisition and analysis. KC, J-JS and SKL contributed in the design of the study, patient management and data acquisition. S-MK contributed in the initial conceptualisation and design of the study, patient management, revised the manuscript and supervised the entire procedures in this study. S-TL contributed in the initial conceptualisation and design of the study, patient management, acquired study data, performed data interpretation, drafted the manuscript, managed the entire study cohort, and is responsible for the overall content as the guarantor.
Funding This work was supported by a National Research Foundation of Korea (NRF) grant funded by the Ministry of Science, ICT and Future Planning, Republic of Korea (2019R1A2C1009003) and by the Korea Health Industry Development Institute, Ministry of Health and Welfare, Republic of Korea (HI21C0539). W-JL and S-TL were supported by Lee Sueng Moon research fund of Seoul National University Hospital (3020170130).
Competing interests S.-TL reports advisory roles for Roche/Genentech, UCB, Biofire Diagnostics, GC Pharma, and Advanced Neural Technologies.
S-MK has lectured, consulted, and received honoraria from Bayer Schering Pharma, Genzyme, Merck Serono, and UCB; received a grant from the National Research Foundation of Korea and the Korea Health Industry Development Institute Research; is an Associate Editor of the Journal of Clinical Neurology. SMK and Seoul National University Hospital has transferred the technology of flow cytometric autoantibody assay to EONE Laboratory, Korea.
Provenance and peer review Not commissioned; externally peer reviewed.
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