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A patient with anti-aquaporin 4 antibody presenting hypersomnolence as the initial symptom and symmetrical hypothalamic lesions.

Abstract
Here we report a case with positive serum anti-aquaporin 4 (AQP4) antibody who presented with hypersomnolence, symmetrical hypothalamic lesions and a reduced CSF orexin (hypocretin) level without optic nerve and spinal cord lesions on MRI. All of the symptoms, MRI finding and CSF orexin level improved simultaneously after steroid therapy. AQP4 is a member of the AQP superfamily which is strongly expressed in the hypothalamus where orexin (hypocretin)-containing neurons are primarily concentrated. Although there have been only a few reports similar to our case, the present case suggests a close relationship between the positive serum anti-AQP4 antibody and symmetrical hypothalamic lesions with hypersomnolence and without optic /spinal lesion, which is improved by steroid treatment.
AuthorsKentaro Deguchi, Syoichiro Kono, Shoko Deguchi, Nobutoshi Morimoto, Masami Ikeda, Tomoko Kurata, Yoshio Ikeda, Tohru Matsuura, Takashi Kanbayashi, Toshiyuki Takahashi, Koji Abe
JournalJournal of the neurological sciences (J Neurol Sci) Vol. 312 Issue 1-2 Pg. 18-20 (Jan 15 2012) ISSN: 1878-5883 [Electronic] Netherlands
PMID21917270 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
CopyrightCopyright © 2011 Elsevier B.V. All rights reserved.
Chemical References
  • AQP4 protein, human
  • Aquaporin 4
  • Autoantibodies
Topics
  • Adult
  • Aquaporin 4 (immunology)
  • Autoantibodies (blood)
  • Diagnosis, Differential
  • Disorders of Excessive Somnolence (diagnosis, immunology, pathology)
  • Female
  • Humans
  • Hypothalamus (immunology, pathology)

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