Area postrema syndrome: Intractable hiccups and vomiting as a result of neuromyelitis Optica Spectrum disorder

Abstract

A 31 year old woman was referred to the medical team for further investigation of intractable hiccups and vomiting. Initial investigations including blood tests, endoscopy and CT imaging did not identify any cause of symptoms. Following multidisciplinary team review, serial MRI Head imaging was arranged, which revealed progressive posterior fossa signal abnormality with involvement of the area postrema. In combination with a positive serum Aquaporin-4 antibody result, this helped establish a diagnosis of Neuromyelitis Optica Spectrum Disorder (NMOSD). Treatment included high dose steroids, plasma exchange and immunomodulatory therapy, and led to a marked improvement in symptoms. This case highlights the importance of utilising specialty team input and broadening lines of investigation, when managing patients with intractable hiccups and vomiting in whom an initial workup has not established a clear diagnosis. While NMOSD is rare, early identification can inform treatment strategies that may lead to a significant improvement in clinical outcome.