Citation, DOI, disclosures and article data
Citation:
Rogalskyi V, Gaillard F, Fahrenhorst-Jones T, et al. Area postrema syndrome. Reference article, Radiopaedia.org (Accessed on 16 Apr 2024) https://doi.org/10.53347/rID-98765
Disclosures:
At the time the article was last revised Frank Gaillard had the following disclosures:
- Biogen Australia Pty Ltd, Investigator-Initiated Research Grant for CAD software in multiple sclerosis: finished Oct 2021 (past)
These were assessed during peer review and were determined to
not be relevant to the changes that were made.
View Frank Gaillard's current disclosures
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.
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.
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.
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T1: hypointense
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T2/FLAIR: hyperintense
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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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1. Shosha E, Dubey D, Palace J et al. Area Postrema Syndrome. Neurology. 2018;91(17):e1642-51. doi:10.1212/wnl.0000000000006392 - Pubmed
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2. Wingerchuk D, Banwell B, Bennett J et al. International Consensus Diagnostic Criteria for Neuromyelitis Optica Spectrum Disorders. Neurology. 2015;85(2):177-89. doi:10.1212/wnl.0000000000001729 - Pubmed
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3. Chan K & Vorobeychik G. Area Postrema Syndrome: A Neurological Presentation of Nausea, Vomiting and Hiccups. BMJ Case Rep. 2020;13(11):e238588. doi:10.1136/bcr-2020-238588 - Pubmed
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4. Cohen D, Craven C, Bragin I. Ischemic Stroke Induced Area Postrema Syndrome With Intractable Nausea, Vomiting, and Hiccups. Cureus. 2020;12(6):e8630. doi:10.7759/cureus.8630 - Pubmed
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5. Zeiner P, Brandhofe A, Müller-Eschner M, Steinmetz H, Pfeilschifter W. Area Postrema Syndrome as Frequent Feature of Bickerstaff Brainstem Encephalitis. Ann Clin Transl Neurol. 2018;5(12):1534-42. doi:10.1002/acn3.666 - Pubmed
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6. Natsis K, Kalyvas A, Theochari E, Papamichalis E, Gerkou A. Area Postrema Syndrome in a Patient with Brainstem Glioblastoma. Acta Neurol Belg. 2021;121(4):1087-8. doi:10.1007/s13760-021-01736-9 - Pubmed
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