Area postrema syndrome

Last revised by Frank Gaillard on 6 Mar 2023

Area postrema syndrome is a disorder of the chemoreceptor trigger zone (area postrema) which is located on the medial posteroinferior surface of the medulla oblongata. It is usually a demyelinating disorder, as one of the core clinical characteristics of neuromyelitis optica spectrum disorder 1,2.

The presentation is with hiccups, nausea, and/or uncontrollable vomiting, typically persisting for at least 48 hours 1-3.

Area postrema syndrome is a classically described manifestation of neuromyelitis optica spectrum disorder, where it is one of the core clinical presentations, because the area postrema is particularly rich in aquaporin-4 1-3. However, the syndrome has also been rarely reported to occur with other pathologies, such as ischemic stroke, brainstem glioma, and Bickerstaff encephalitis 4-6.

MRI is the modality of choice for area postrema syndrome.

Typical appearance of area postrema syndrome is a V-shaped circumventricular demyelinating zone located on the posteroinferior surface of the medulla oblongata at the caudal end of the fourth ventricle 2,3.

  • T1: hypointense
  • T2/FLAIR: hyperintense
  • T1 C+ (Gd): enhancement is variable

When occurring due to neuromyelitis optica spectrum disorder, high-dose methylprednisolone is the treatment of choice initially.

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Cases and figures

  • Case 1
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  • Case 2: area postrema syndrome
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  • Case 3
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