Case Report: AQP4-IgG-positive neuromyelitis optica spectrum disorder with CSF anti- NMDAR1 IgG positivity and encephalitic features
By
Mingming Li
Lixia Chen
Yanjun Liu
Tiejun Gan
Jia Guo
July 13, 2026
Clinical Scorecard: Clinical Case: Neuromyelitis Optica Spectrum Disorder Positive for AQP4-IgG with Concurrent CSF Anti-NMDAR1 IgG and Encephalitic Symptoms
At a Glance
Category Detail
Condition Neuromyelitis Optica Spectrum Disorder (NMOSD) Key Mechanisms AQP4-IgG positivity and potential overlap with anti-NMDAR encephalitis Target Population Adults with NMOSD and possible anti-NMDAR encephalitis overlap Care Setting Neurology and immunology clinical settings
Key Highlights
AQP4-IgG-positive NMOSD can present with brain-predominant lesions. Overlap with anti-NMDAR encephalitis is uncommon but possible. CSF analysis showed positive AQP4-IgG and low titer anti-NMDAR1 IgG. Immunotherapy led to substantial improvement in cognitive and visual function. Clinical correlation of symptoms, MRI findings, and antibody results is crucial. Guideline-Based Recommendations
Diagnosis
Consider NMOSD in patients with brain lesions and atypical symptoms. Evaluate for anti-NMDAR antibodies in cases of cognitive decline with NMOSD. Management
Initiate high-dose intravenous methylprednisolone for acute treatment. Follow with oral prednisone taper and maintenance therapy with inebilizumab. Monitoring & Follow-up
Regular follow-up MRI to assess for new lesions. Cognitive and visual function assessments during follow-up. Risks
Potential for misdiagnosis as nutritional encephalopathy. Risk of delayed treatment in cases of overlapping syndromes. Patient & Prescribing Data
Adults with AQP4-IgG-positive NMOSD and neuropsychiatric symptoms.
High-dose steroids followed by inebilizumab can improve symptoms.
Clinical Best Practices
Correlate clinical symptoms with MRI findings and antibody results. Consider autoimmune overlap syndromes in atypical presentations. Related Resources & Content
