• Online Features  This Article  • Full text  • PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on Web of Science (8)  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Topic Collections  • Neurology  • Multiple Sclerosis/ Demyelinating Disease  • Neuro-ophthalmology  • Neurology, Other  • Immunologic Disorders  • Alert me on articles by topic  Social Bookmarking   What's this?

New Neurochemical and Immunological Implications

Takashi Kanbayashi, MD, PhD; Takayoshi Shimohata, MD, PhD; Ichiro Nakashima, MD, PhD; Hiroaki Yaguchi, MD; Ichiro Yabe, MD, PhD; Masatoyo Nishizawa, MD, PhD; Tetsuo Shimizu, MD, PhD; Seiji Nishino, MD, PhD

Arch Neurol. 2009;66(12):1563-1566.

Objective  To characterize factors that contribute to symptomatic narcolepsy and excessive daytime sleepiness in neuromyelitis optica and multiple sclerosis.

Setting  Japanese university hospitals.

Design  Case study.

Patients  Seven Japanese patients whose initial diagnoses were multiple sclerosis and who were exhibiting excessive daytime sleepiness.

Main Outcome Measures  Lesions on magnetic resonance imaging, cerebrospinal fluid hypocretin-1 levels, and serum anti–aquaporin 4 (AQP4) antibody titer.

Results  Bilateral and symmetrical hypothalamic lesions associated with marked or moderate hypocretin deficiency were found in all 7 cases. Four of these patients met the International Classification of Sleep Disorders 2 narcolepsy criteria. Three patients, including 2 patients with narcolepsy, were seropositive for anti-AQP4 antibody and diagnosed as having neuromyelitis optica–related disorder.

Conclusion  Since AQP4 is highly expressed in the hypothalamic periventricular regions, an immune attack on AQP4 may be partially responsible for the bilateral and hypothalamic lesions and hypocretin deficiency in narcolepsy/excessive daytime sleepiness associated with autoimmune demyelinating diseases.

Author Affiliations: Department of Neuropsychiatry, Akita University School of Medicine, Akita City (Drs Kanbayashi and Shimizu), Department of Neurology, Brain Research Institute, Niigata University, Niigata (Drs Shimohata and Nishizawa), Department of Neurology, Tohoku University School of Medicine, Sendai (Dr Nakashima), and Department of Neurology, Hokkaido University Graduate School of Medicine, Sapporo (Drs Yaguchi and Yabe), Japan; and Department of Psychiatry and Behavioral Sciences, Stanford University School of Medicine, Palo Alto, California (Dr Nishino).

CiteULike    Connotea    Delicious    Digg    Facebook    Reddit    Technorati    Twitter     What's this?