Neuromyelitis optica spectrum disorder (NMOSD): area postrema syndrome (APS)

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Neuromyelitis optica spectrum disorder (NMOSD): area postrema syndrome (APS). This is caused by dorsal medullar lesions.

Pathogenesis

  • This is caused by dorsal medullar lesions
  • The lesion may extend to the upper cervical cord
  • It occurs in aquaporin-4 (AQP4) antibody positive NMOSD

Epidemiological features

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Classic clinical triad

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Pathological features

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Other causes of area postrema syndrome

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References

  1. Shosha E, Dubey D, Palace J, et al. Area postrema syndrome: frequency, criteria, and severity in AQP4-IgG-positive NMOSD. Neurology 2018; 91:e1642-e1651. 
  2. You XF, Hu HT, Ye J. Characteristics of neuromyelitis optica spectrum disorder associated with the area postrema. Zhonghua Yi Xue Za Zhi 2018; 98:668-672. 
  3. Popescu BF, Lennon VA, Parisi JE, et al. Neuromyelitis optica unique area postrema lesions: nausea, vomiting, and pathogenic implications. Neurology 2011; 76:1229-1237. 
  4. Camara-Lemarroy CR, Burton JM. Area postrema syndrome: a short history of a pearl in demyelinating diseases. Mult Scler 2019; 25:325-329.
  5. Ciron J, Sourdrille F, Biotti D, et al. Area postrema syndrome: another feature of anti-GFAP encephalomyelitis. Mult Scler 2019; doi: 10.1177/1352458518817992 (Epub ahead of print).
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