Abstract |
Recent studies have demonstrated that hypothalamic lesions associated with brain tumor, head trauma, and encephalopathy can cause symptomatic hypersomnia with a reduced orexin ( hypocretin) level in the cerebrospinal fluid (CSF). Aquaporin 4 (AQP4), a member of the AQP superfamily, is strongly expressed in the hypothalamus in which orexin ( hypocretin)-containing neurons are primarily concentrated. We report the case of a patient with a serum anti-AQP4 antibody who presented with recurrent hypersomnia, symmetrical hypothalamic lesions with long spinal cord lesions on MRI, and a reduced CSF orexin ( hypocretin) level, all of which were improved simultaneously by steroid therapy. Further studies should be performed to determine the roles of anti-AQP4 antibody positivity in patients with hypersomnia associated with orexin ( hypocretin) deficiency and hypothalamic lesions.
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Authors | Hiroaki Nozaki, Takayoshi Shimohata, Takashi Kanbayashi, Youhei Sagawa, Shin-Ichi Katada, Masahisa Satoh, Osamu Onodera, Keiko Tanaka, Masatoyo Nishizawa |
Journal | Sleep medicine
(Sleep Med)
Vol. 10
Issue 2
Pg. 253-5
(Feb 2009)
ISSN: 1389-9457 [Print] Netherlands |
PMID | 18226957
(Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- AQP4 protein, human
- Antibodies
- Aquaporin 4
- Intracellular Signaling Peptides and Proteins
- Neuropeptides
- Orexins
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Topics |
- Adult
- Antibodies
(blood)
- Aquaporin 4
(immunology)
- Cervical Vertebrae
- Disorders of Excessive Somnolence
(metabolism, pathology)
- Female
- Humans
- Hypothalamus
(pathology)
- Intracellular Signaling Peptides and Proteins
(cerebrospinal fluid)
- Neuropeptides
(cerebrospinal fluid)
- Orexins
- Spinal Cord
(pathology)
- Thoracic Vertebrae
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