A 36-year-old woman presented with hemiplegia and loss of pain and temperature sensation 1 day after the subacute onset of intractable vomiting. Paresthesia followed. She was tetraplegic at day 12. Initial MRI revealed a lesion in the medulla oblongata, involving primarily the area postrema (figure). The lesion progressively extended into the upper cervical cord. Forebrain MRI was normal. CSF contained 11 leukocytes/mm3. Brainstem tumor and multiple sclerosis were early diagnostic considerations. Aquaporin-4 immunoglobulin (Ig)G was detected in serum. This case supports the concept that fenestrated capillaries in the area postrema are an important initial CNS entry site for pathogenic neuromyelitis optica–IgG in neuromyelitis optica spectrum disorders.1,2
Signal abnormality starting in the fourth ventricle floor (area postrema; day 1) evolves over the next 10 days to upper cervical transverse myelitis. The aquaporin-4–rich area postrema, which is the vomiting center of the medulla oblongata, lacks a blood-brain barrier. Sagittal images, T2-weighted (without contrast).
Footnotes
Author contributions: N. Asgari: study concept and design, acquisition and interpretation of data, writing of draft manuscript. H.P.B. Skejoe: MRI reevaluation and manuscript revision. V.A. Lennon: discussion of case and immunopathogenic implications and revision of manuscript.
Study funding: No targeted funding reported.
Disclosure: N. Asgari and H.P.B. Skejoe report no disclosures. Mayo Clinic holds a patent relating to aquaporin-4 as a target of pathogenic autoantibodies in neuromyelitis optica and related disorders. V.A. Lennon has received royalties exceeding the federal threshold for significant financial interest from the licensing of this technology and has rights to receive future royalties. In addition, V.A. Lennon is a named inventor on 2 patent applications filed by Mayo Foundation for Medical Education and Research relating to functional assays for detecting aquaporin-4 IgG and applications to cancer. She receives no revenue from the sale of serologic tests performed by Mayo Medical Laboratories. Go to Neurology.org for full disclosures.
- © 2013 American Academy of Neurology
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