Clinical and imaging features and treatment response of anti-NMDAR encephalitis combined with MOGAD
Guanhua Yan1, De-Cai Tian2, Xinghu Zhang2
1Departments of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China; Departments of Neurology, Liaocheng Peoples' Hospital, Liaocheng 252000, China.
Background:
To investigate the clinical, imaging features, immunotherapy of anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) combined with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).
Methods:
A total of 124 patients with NMDARE and 86 patients with MOGAD were screened from an ongoing prospective cohort study (Clinical and Imaging Patterns of Neuroinflammation Diseases in China, CLUE). Ten NMDARE combined with MOGAD patients, were finally enrolled in this study. Clinical and imaging data and follow-up results characteristics were collected and analyzed.
Results:
In these 10 patients with NMDARE combined with MOGAD, 7 patients (70 %) showed recurrent courses. In all 26 episodes, 14 episodes (53.8 %) showed encephalitis-related symptoms, 6 episodes (23.1 %) showed demyelination-related symptoms, 6 episodes (23.1 %) showed both. The median CSF leukocytes were 13/μL (range 1-413) and the median protein was 0.43 g/L (range 0.22-0.70). MRI lesions were found involving the optic nerve (2/10), spinal cord (3/10), deep gray matter (3/10), cortex (6/10), subcortex (7/10), brainstem (5/10) and cerebellum (4/10). Leptomeningeal enhancement was found in 3 patients. All patients received high-dose intravenous methylprednisolone and immunoglobulin therapy during attacks. Seven patients received rituximab (RTX). The median annualized recurrence rate (ARR) reduced significantly following RTX treatments (z = -2.201, p = 0.028), and achieved good outcomes at the last follow-up visit (modified Rankin scale score ≤ 2).
Conclusion:
NMDARE combined with MOGAD represents a unique characteristic of autoantibody-mediated encephalitis. Coexistence of NMDAR and MOG antibody may indicate high recurrence risk. RTX may be a relatively efficient therapeutic option.
Insights
Anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) co-occurrence presents unique challenges. Rituximab (RTX) significantly reduced recurrence rates in these patients, suggesting its therapeutic efficacy.
Area of Science:
- Neurology
- Immunology
- Neuroimmunology
Background:
- Anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are distinct autoimmune neurological disorders.
- Investigating the combined presentation of NMDARE and MOGAD is crucial for understanding complex neuroinflammatory conditions.
Purpose of the Study:
- To characterize the clinical and imaging features of patients with coexisting NMDARE and MOGAD.
- To evaluate the effectiveness of immunotherapy, particularly rituximab (RTX), in managing this combined condition.
Main Methods:
- A prospective cohort study identified 10 patients with both NMDARE and MOGAD.
- Clinical data, neuroimaging findings, and treatment outcomes were analyzed.
- Cerebrospinal fluid (CSF) analysis and modified Rankin scale (mRS) scores were assessed.
Main Results:
- Seven out of ten patients experienced recurrent disease courses.
- Symptoms included encephalitis (53.8%), demyelination (23.1%), or both (23.1%).
- MRI revealed lesions in various brain regions; RTX treatment significantly reduced the annualized recurrence rate (ARR) and led to favorable outcomes (mRS ≤ 2).
Conclusions:
- The combination of NMDARE and MOGAD represents a distinct entity within autoantibody-mediated encephalitis.
- Coexistence of NMDAR and MOG antibodies is associated with a high risk of recurrence.
- Rituximab (RTX) demonstrates potential as an effective therapeutic option for this combined condition.
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