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Research Letter
January 09, 2017

Association of Extension of Cervical Cord Lesion and Area Postrema Syndrome With Neuromyelitis Optica Spectrum Disorder

Author Affiliations
  • 1Department of Neurology, Mayo Clinic, Rochester, Minnesota
  • 2Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota
  • 3Department of Radiology, Mayo Clinic, Rochester, Minnesota
JAMA Neurol. Published online January 9, 2017. doi:10.1001/jamaneurol.2016.5441

Neuromyelitis optica spectrum disorders (NMOSDs) are autoimmune disorders of astrocytic water channels associated with the highly specific serum biomarker, aquaporin-4 (AQP-4) IgG.1 Aquaporin-4 has high expression in the circumventricular organs and spinal cord. The area postrema (AP), located in the dorsal medulla, is the chemosensitive vomiting center and has high AQP-4 expression.2 The AP syndrome with unexplained hiccups, nausea, and vomiting is one of the core clinical characteristics in the 2015 diagnostic criteria for NMOSDs and may occur concurrently with a myelitis episode.1 Prior studies have suggested spinal cord lesions extending to the AP are specific for NMOSDs.3 However, in our clinical practice, we have encountered numerous non-NMOSD long myelopathies extending to the region of the AP. In this study, we evaluated whether longitudinally extensive cervical cord lesions extending to or accompanied by AP/dorsal medulla lesions on magnetic resonance imaging (MRI) are specific for NMOSDs.

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