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

13 Jul 20264 min read0 viewsJournal Feed

GIST (Key Takeaways)

Background. Overlap between aquaporin-4 immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD) and anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is uncommon and may be difficult to recognize, especially when NMOSD initially presents with brain-predominant lesions and nonspecific symptoms. Case presentationA 47-year-old woman initially presented with anorexia, dizziness, nausea, and intractable vomiting.
Brain MRI showed a non-enhancing lesion along the anterior wall of the third ventricle involving the hypothalamic region and adjacent periependymal diencephalon, and she was initially treated for possible Wernicke encephalopathy with only partial transient improvement. Seven months later, she developed bilateral blurred vision, and repeat MRI showed persistent diencephalic involvement together with a new lesion in the left cerebellar hemisphere. Over the following three months, she developed progressive cognitive decline and neuropsychiatric symptoms.
Neuro-ophthalmologic assessment supported bilateral optic neuritis. Brain MRI later revealed bilateral thalamic and mesial temporal abnormalities, while cervical MRI disclosed a subtle dorsal medullary lesion corresponding to the area postrema. Cerebrospinal fluid (CSF) showed no pleocytosis or protein elevation, but serum and CSF were positive for AQP4-IgG, and CSF anti-NMDAR1 IgG was positive at a low titer (1:10).

Clinical Editorial

Frontiers in Immunology published a clinical update in Infectious Disease on 13 Jul 2026.

The item focuses on Case Report: AQP4-IgG-positive neuromyelitis optica spectrum disorder with CSF anti- NMDAR1 IgG positivity and encephalitic features.

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