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Updated: Jun 18, 2025
Induction and Diverse Assessment Indicators of Experimental Autoimmune Encephalomyelitis
Published on: September 9, 2022
NMDAR autoimmune encephalitis and fulminant relapse of multiple sclerosis: a rare overlap syndrome
Saxon Douglass1, Deborah Field2
1Department of Neurology, Royal Adelaide Hospital, Adelaide, SA, Australia saxon@saxondouglass.com.
Abstract:
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disease that has been rarely associated with AQP4-IgG and MOG-IgG demyelinating diseases, and even more rarely with multiple sclerosis. We present the case of a woman in her 40s with confirmed NMDAR encephalitis and coexistent fulminant relapse of multiple sclerosis treated with alemtuzumab 6 years prior, who had a favourable outcome following treatment with ocrelizumab. We proceed to systematic review of similar reported cases, finding a lower than anticipated prevalence of underlying malignancy compared with isolated NMDAR encephalitis, in this rare overlap syndrome.
Insights
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis rarely co-occurs with multiple sclerosis. This case study and review highlight favorable outcomes with ocrelizumab treatment in this rare overlap syndrome, with less frequent malignancy than expected.
Area of Science:
- Neuroimmunology
- Neurology
- Autoimmune Diseases
Background:
- Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a rare autoimmune neurological disorder.
- Co-occurrence with demyelinating diseases like multiple sclerosis (MS) is exceptionally uncommon.
- Previous associations with AQP4-IgG and MOG-IgG are rare, and with MS even rarer.
Purpose of the Study:
- To report a case of NMDAR encephalitis coexisting with a severe relapse of multiple sclerosis.
- To investigate the clinical course and treatment outcomes in this rare overlap syndrome.
- To systematically review similar reported cases to understand prevalence and associated conditions.
Main Methods:
- Case presentation of a patient with confirmed NMDAR encephalitis and a history of fulminant MS relapse.
- Literature review of reported cases of coexistent NMDAR encephalitis and multiple sclerosis.
- Analysis of treatment strategies and patient outcomes, including malignancy prevalence.
Main Results:
- The patient with NMDAR encephalitis and multiple sclerosis experienced a favorable outcome after treatment with ocrelizumab.
- Systematic review revealed a lower prevalence of underlying malignancy in this overlap syndrome compared to isolated NMDAR encephalitis.
- Alemtuzumab treatment for MS 6 years prior did not preclude a favorable response to ocrelizumab.
Conclusions:
- Ocrelizumab may be an effective treatment option for patients with the rare overlap syndrome of NMDAR encephalitis and multiple sclerosis.
- The co-occurrence of NMDAR encephalitis and multiple sclerosis appears to have a different risk profile regarding malignancy compared to NMDAR encephalitis alone.
- This rare overlap syndrome warrants further investigation to optimize management strategies.
