Case Report: AQP4-IgG-positive neuromyelitis optica spectrum disorder with CSF anti- NMDAR1 IgG positivity and encephalitic features - Takeaways - MDSpire

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

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  • 1

    A 47-year-old woman with AQP4-IgG-positive NMOSD initially presented with nonspecific symptoms and brain lesions.

  • 2

    Her condition progressed to cognitive decline and neuropsychiatric symptoms, complicating the diagnosis.

  • 3

    CSF analysis revealed positive AQP4-IgG and low titer anti-NMDAR1 IgG, indicating a potential overlap of conditions.

  • 4

    The patient was treated with high-dose intravenous methylprednisolone and inebilizumab, leading to significant improvement.

  • 5

    This case emphasizes the importance of correlating clinical symptoms with MRI and antibody findings in autoimmune disorders.

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