Case Report: AQP4-IgG-positive neuromyelitis optica spectrum disorder with CSF anti- NMDAR1 IgG positivity and encephalitic features - Report - MDSpire
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Case Report: AQP4-IgG-positive neuromyelitis optica spectrum disorder with CSF anti- NMDAR1 IgG positivity and encephalitic features
Clinical Case: Neuromyelitis Optica Spectrum Disorder Positive for AQP4-IgG
Background
Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune condition characterized by the presence of AQP4-IgG antibodies, leading to severe neurological symptoms. The overlap between NMOSD and anti-NMDAR encephalitis is rare and can complicate diagnosis and treatment, particularly when initial symptoms are nonspecific. Understanding these overlaps is essential for accurate diagnosis.
Data Highlights
No numerical data or trial data presented in the article.
Key Findings
A 47-year-old woman presented with symptoms including anorexia, dizziness, and vomiting.
Brain MRI revealed a lesion in the anterior wall of the third ventricle, initially misdiagnosed as Wernicke encephalopathy.
CSF analysis showed positive AQP4-IgG and low-titer anti-NMDAR1 IgG.
The patient exhibited cognitive decline and neuropsychiatric symptoms, with MRI showing bilateral thalamic and mesial temporal abnormalities.
High-dose intravenous methylprednisolone followed by inebilizumab treatment led to substantial improvement in cognitive and visual functions.
Clinical Implications
Clinicians should consider NMOSD in patients presenting with encephalitic symptoms and atypical MRI findings. Vigilance for potential overlaps between NMOSD and anti-NMDAR encephalitis is necessary for accurate diagnosis.
Conclusion
This case illustrates the complexity of diagnosing autoimmune overlap syndromes and the importance of correlating clinical, imaging, and laboratory findings.
