Intractable Vomiting as an Initial and Evasive Presentation of Neuromyelitis Optica Spectrum Disorder

顽固性呕吐是视神经脊髓炎谱系障碍的首发且难以诊断的表现

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Abstract

Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon autoimmune neurological condition that is rarely associated with vomiting as the only initial symptom. We report a 67-year-old Caribbean female who was admitted to the general medicine ward with intractable vomiting for about two weeks. Her condition two weeks later progressed to brainstem syndrome, progressive dysphagia, and slurred speech. A month from admission, she developed quadriparesis, symptoms initially suggestive of a stroke. The underlying neurological diagnosis remained unclear for some time. Her initial symptoms were attributed to viral gastroenteritis, later with gastroscopy to esophagitis and subsequently to a possible stroke, which brain CT and MRI ruled out. However, a brain MRI with contrast revealed longitudinal and extensive hyperintense T2 changes in the brainstem, including the area postrema (lower medulla) and the cervical region. Ultimately, after more than a month of her admission, she was diagnosed with NMOSD, confirmed by the presence of aquaporin-4 antibodies in the cerebrospinal fluid via lumbar puncture. She received high-dose corticosteroid therapy and plasma exchange as acute treatment, followed by maintenance with mycophenolate. This led to improvements in her speech and swallowing; however, the improvement in her muscle weakness was not that prominent. After a long hospital admission, a patient was transferred to a neurorehabilitation centre to support recovery and enhance muscle strength, along with immunosuppressive treatment. During her extended hospital stay, the patient required nasogastric feeding, and her course was complicated by recurrent chest and urinary tract infections. This case highlights the atypical presentation and diagnostic challenges of NMOSD. We hope this report will help clinicians consider NMOSD earlier in similar clinical scenarios.

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