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Myelin Oligodendrocyte Glycoprotein MOG35-55 Induced Experimental Autoimmune Encephalomyelitis EAE in C57BL/6 Mice
Published on: April 15, 2014
MOG antibody-associated encephalitis in adult: clinical phenotypes and outcomes
Woo-Jin Lee1,2, Young Nam Kwon1, Boram Kim1
1Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, South Korea.
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.
Insights
Myelin oligodendrocyte glycoprotein antibody-associated autoimmune encephalitis (MOGAE) outcomes vary by subtype. Short immunotherapy maintenance and brain atrophy correlate with relapse and poor outcomes, especially in dual MOG-antibody and NMDAR-antibody patients.
Area of Science:
- Neurology
- Immunology
- Neuroscience
Background:
- Investigating clinical characteristics and outcomes of MOG antibody-associated autoimmune encephalitis (MOGAE) in adult patients.
- Focus on institutional cohort with over 1-year follow-up.
Purpose of the Study:
- To analyze disease severity, immunotherapy, outcomes, and relapses in adult MOGAE patients.
- To evaluate serial brain MRI data in relation to clinical course.
Main Methods:
- Analysis of 40 adult MOGAE patients.
- Assessment of disease severity using modified Rankin Scale (mRS) and Clinical Assessment Scale in Autoimmune Encephalitis.
- Evaluation of immunotherapy, outcomes, relapses, and serial brain MRI.
Main Results:
- 80% achieved good outcomes (mRS 0-2), 40% relapsed.
- Limbic encephalitis (LE) subtype showed older onset and poorer outcomes but low relapse rate.
- Short immunotherapy (<6 months) linked to 84% of relapses.
- Brain atrophy correlated with poor outcomes.
- Dual MOG-antibody and NMDAR-antibody patients had atypical presentations and higher relapse rates.
Conclusions:
- MOGAE outcomes differ significantly across cortical encephalitis, LE, and ADEM phenotypes.
- Inadequate immunotherapy maintenance (<6 months) increases relapse risk.
- Brain atrophy on MRI predicts poor clinical outcomes.
- Co-occurrence of MOG- and NMDAR-antibodies is associated with high relapse rates.
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