Area Postrema Syndrome Presenting as Intractable Hiccups and Vomiting: An Isolated Initial Manifestation of Aquaporin-4 Positive Neuromyelitis Optica Spectrum Disorder – A Case Report

Abstract

Background: Neuromyelitis optica spectrum disorder (NMOSD) is a potentially disabling autoimmune inflammatory disease of the central nervous system that is marked by the occurrence of recurrent attacks in the optic nerves, spinal cord, brainstem, and periventricular regions. Persistent nausea, vomiting and hiccups are one of the central clinical features of NMOSD, which is often misdiagnosed due to gastrointestinal symptoms being nonspecific, and termed area postrema syndrome (APS).

Case Presentation: A 30-year-old woman presented with three weeks of intractable hiccups and persistent vomiting, but no neurological deficits. The gastrointestinal investigations were normal except for significant length. Brain magnetic resonance imaging (MRI) revealed a hyperintense lesion on T2-weighted images in the area of the dorsal medulla and involving the area postrema. Immunoglobulin G (IgG) aquaporin-4 (AQP4) was strongly positive in serum testing. MRI of the spinal cord and ophthalmological examinations were normal. The patient had the initial symptoms of NMOSD as isolated APS. The complete clinical recovery observed at 12 months follow-up was obtained with high dose intravenous methylprednisolone and oral corticosteroid taper plus rituximab.

Conclusion: APS could be the first and the purest form of AQP4-positive NMOSD. Early diagnosis and immediate immunotherapy are key to avoid permanent neurological damage. In a patient with unknown persistent hiccups, vomiting post exclusion of gastrointestinal causes, NMOSD should be considered.