Abstract
Objectives: To compare the frequency of area postrema syndrome (APS) in adults with anti-aquaporin-4 (AQP4) and anti-myelin oligodendrocyte glycoprotein (MOG) antibodies.Methods: APS is defined as acute or subacute, single or combined, episodic or constant nausea, vomiting, or hiccups, persisting for at least 48 h, which cannot be attributed to any other etiology. The presence of APS was investigated in 274 adults with AQP4 antibodies and 107 adults with MOG antibodies from 10 hospitals.Results: The study population comprised Korean adults (≥18 years). At the time of disease onset, 14.9% (41/274) adults with AQP4 antibodies had APS, while none of the participants with MOG antibodies developed APS (p < 0.001). During the course of the disease, 17.2% (47/274) adults with AQP4 antibodies had APS in contrast to 1.9% (2/107) adults with MOG antibodies with APS (p < 0.001).Conclusions: APS, one of the core clinical characteristics of individuals with AQP4 antibodies, is an extremely rare manifestation in Korean adults with MOG antibodies.
Highlights
At the time of disease onset, 14.9% (41/274) adults with AQP4 antibodies had AQP4 vs. MOGArea postrema syndrome (APS), while none of the participants with myelin oligodendrocyte glycoprotein (MOG) antibodies developed APS (p < 0.001)
During the course of the disease, 17.2% (47/274) adults with AQP4 antibodies had APS in contrast to 1.9% (2/107) adults with MOG antibodies with APS (p < 0.001)
Individuals with anti-aquaporin-4 (AQP4) antibodies and anti-myelin oligodendrocyte glycoprotein (MOG) antibodies were previously grouped under the umbrella term neuromyelitis optica spectrum disorder (NMOSD), as they shared two cardinal clinical manifestations, optic neuritis and longitudinally extensive transverse myelitis
Summary
Individuals with anti-aquaporin-4 (AQP4) antibodies and anti-myelin oligodendrocyte glycoprotein (MOG) antibodies were previously grouped under the umbrella term neuromyelitis optica spectrum disorder (NMOSD), as they shared two cardinal clinical manifestations, optic neuritis and longitudinally extensive transverse myelitis. Area postrema syndrome (APS), one of the core clinical characteristics described in the 2015 diagnostic criteria for NMOSD, is defined as intractable nausea, vomiting, or hiccups, which persist for at least 48 h [4, 5]. In contrast to the preferential expression of AQP4 in certain regions of the central nervous system (CNS) [9], MOG is expressed throughout the CNS. APS is not expected in individuals with MOG antibodies, unlike those with AQP4 antibodies. This study evaluated the value of APS as a clinical characteristic in differentiating individuals with AQP4 antibodies from those with MOG antibodies in a large Korean cohort
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