2018
DOI: 10.1212/wnl.0000000000006392
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Area postrema syndrome

Abstract: ObjectiveTo define the frequency, duration, and severity of intractable nausea, vomiting, or hiccups in aquaporin-4–immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD) and propose diagnostic criteria and a severity scale for area postrema syndrome (APS).MethodsAn International NMOSD database was interrogated for frequency of APS. Patients with AQP4-IgG–positive NMOSD completed an APS symptom questionnaire. Nausea and vomiting severity was derived from the Pregnancy-Unique Quanti… Show more

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Cited by 138 publications
(76 citation statements)
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“…We found that area postrema relapses were more common at first presentation than with subsequent relapses. This finding is contrary to a recent larger study of several international cohorts (16). However, we note the definition for area postrema syndrome used in that study was broader than the definition used in the present study.…”
Section: Discussioncontrasting
confidence: 99%
“…We found that area postrema relapses were more common at first presentation than with subsequent relapses. This finding is contrary to a recent larger study of several international cohorts (16). However, we note the definition for area postrema syndrome used in that study was broader than the definition used in the present study.…”
Section: Discussioncontrasting
confidence: 99%
“…The AQP-4 antibody binding triggers complement activation and subsequently the downstream effects and histopathology seen in NMSOD. Patients also commonly present with blindness and paralysis due to AQP-4 protein density on optic nerves and spinal cord, whereas isolated APS attacks in AQP-4-positive NMOSD have a prevalence of 7.1-14.5% [6,7].…”
Section: Discussionmentioning
confidence: 99%
“…Area postrema syndrome/lesions have been identified as disease-defining symptoms for AQP4-positive NMOSD (13,14); however, this syndrome/localization is not specific (14). In Case I, we observed extensive myelitis extending from the dorsal medulla to C6; however, the patient's history was unremarkable for intractable nausea, vomiting, or hiccups.…”
Section: Discussionmentioning
confidence: 79%