Abstract
Though neuromyelitis optica spectrum disorder (NMOSD) has been traditionally characterized by recurrent attacks of optic neuritis and myelitis, the phenotype of this disorder has expanded in recent years, and patients may present with brain lesions in regions where aquaporin 4 is highly expressed, such as the hypothalamus and area postrema. When the area postrema is involved, patients can develop intractable nausea and vomiting or hiccups. These patients should be evaluated with complete neuroimaging of the brain and spinal cord to determine the presence of other areas of inflammation, and anti-aquaporin 4 immunoglobulin should be measured. If a diagnosis of NMOSD is confirmed, additional autoantibodies should be tested as these patients often have comorbid autoimmune disorders. Additionally, preventative treatment options should be discussed and implemented expediently as attacks in NMOSD can be severe and disabling with poor recovery.
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Narula, S., Waldman, A.T. (2017). Neuromyelitis Optica Spectrum Disorder with Brainstem Presentation. In: Waubant, E., Lotze, T. (eds) Pediatric Demyelinating Diseases of the Central Nervous System and Their Mimics. Springer, Cham. https://doi.org/10.1007/978-3-319-61407-6_24
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DOI: https://doi.org/10.1007/978-3-319-61407-6_24
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