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.
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Clinical presentation
The presentation is with hiccups, nausea, and/or uncontrollable vomiting, typically persisting for at least 48 hours 1-3.
Pathology
Etiology
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.
Radiographic features
MRI is the modality of choice for area postrema syndrome.
MRI
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
Treatment and prognosis
When occurring due to neuromyelitis optica spectrum disorder, high-dose methylprednisolone is the treatment of choice initially.
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