title: Symptomatic narcolepsy in patients with neuromyelitis optica and multiple sclerosis: new neurochemical and immunological implications.
abstract: OBJECTIVE
  To characterize factors that contribute to symptomatic narcolepsy and excessive daytime sleepiness in neuromyelitis optica and multiple sclerosis.
  
  
  METHODS
  Japanese university hospitals.
  
  
  METHODS
  Case study.
  
  
  METHODS
  Seven Japanese patients whose initial diagnoses were multiple sclerosis and who were exhibiting excessive daytime sleepiness.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neuropsychiatry, Akita University School of Medicine, Akita City, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Kanbayashi
authors.forename: Takayoshi
authors.initials: T
authors.lastname: Shimohata
authors.forename: Ichiro
authors.initials: I
authors.lastname: Nakashima
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Yaguchi
authors.forename: Ichiro
authors.initials: I
authors.lastname: Yabe
authors.forename: Masatoyo
authors.initials: M
authors.lastname: Nishizawa
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Shimizu
authors.forename: Seiji
authors.initials: S
authors.lastname: Nishino
mesh_terms: D000328:Adult; D051401:Aquaporin 4 / Q000097:blood; D016022:Case-Control Studies; D005260:Female; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins / Q000134:cerebrospinal fluid; D008297:Male; D008875:Middle Aged; D009103:Multiple Sclerosis / Q000150:complications / Q000276:immunology* / Q000378:metabolism*; D009290:Narcolepsy / Q000150:complications / Q000276:immunology* / Q000378:metabolism*; D009471:Neuromyelitis Optica / Q000150:complications / Q000276:immunology* / Q000378:metabolism*; D009479:Neuropeptides / Q000134:cerebrospinal fluid; D000068797:Orexins; D055815:Young Adult
chemical_list: C496030:AQP4 protein, human; D051401:Aquaporin 4; C000596229:HCRT protein, human; D047908:Intracellular Signaling Peptides and Proteins; D009479:Neuropeptides; D000068797:Orexins