Area postrema syndrome
Citation, DOI & article data
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
Treatment and prognosis
When occurring due to neuromyelitis optica spectrum disorder, high-dose methylprednisolone is the treatment of choice initially.
- 1. Shosha E, Dubey D, Palace J et al. Area Postrema Syndrome. Neurology. 2018;91(17):e1642-51. doi:10.1212/wnl.0000000000006392 - Pubmed
- 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
- 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
- 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
- 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
- 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