Intractable Nausea and Vomiting: A Key Feature of Neuromyelitis Optica Spectrum Disorder

Objective:Rare disease Background:Area postrema syndrome (APS) can present with hiccups, nausea, or vomiting as a neurological presentation of neuromyelitis optica spectrum disorder (NMOSD).However, the diagnosis may be delayed and can result in neurological sequelae that include optic neuritis or myelitis.This report describes the case of a 22-year-old woman with intractable nausea, vomiting, and hiccups due to APS as a clinical manifestation of NMOSD. Case Report:A 22-year-old woman presented with a 5-week history of nausea, vomiting, and hiccups.After 3 emergency department visits, a first admission attributed her symptoms to gastroparesis on the basis of a gastric emptying study showing delayed emptying.Symptoms persisted despite antiemetic and prokinetic therapy, and she was readmitted 2 weeks later with new persistent hiccups and progressive bilateral upper-limb weakness.Magnetic resonance imaging (MRI) of the head and cervical spine demonstrated an expansile T2-hyperintense lesion of the dorsal lower medulla and upper cervical cord.Given a high suspicion for a demyelinating attack, plasma exchange and high-dose intravenous corticosteroids were started before serology results returned.Serum aquaporin-4 immunoglobulin G (AQP4-IgG) was subsequently positive, establishing AQP4-IgG-positive NMOSD.Motor function improved after 7 plasma exchange sessions and rehabilitation, and she regained ambulation with a walker. Conclusions:This report describes APS as the initial manifestation of NMOSD in a young woman whose intractable nausea, vomiting, and hiccups were first attributed to gastroparesis.NMOSD should be considered when unexplained nausea, vomiting, or hiccups persist beyond 48 hours, since prompt MRI and AQP4-IgG testing allow immunotherapy to begin without delay.