title: Cerebrospinal Fluid Orexin A Levels and Autonomic Function in Kleine-Levin Syndrome.
abstract: OBJECTIVE
  Kleine-Levin syndrome (KLS) is a rare disorder of relapsing sleepiness. The hypothesis was that the syndrome is related to a change in the vigilance peptide orexin A.
  
  
  METHODS
  From 2002 to 2013, 57 patients with relapsing hypersomnolence were clinically assessed in a referral academic center in Beijing, China, and 44 (28 males and 16 females; mean age 18.3 ± 8.9 y (mean ± standard deviation, range 9-57 y) were determined to have clinical and behavioral criteria consistent with KLS. Cerebrospinal fluid orexin A levels and diurnal blood pressure were measured in relapse versus remission in a subgroup of patients.
  
  
  RESULTS
  Presenting symptoms included relapsing or remitting excessive sleepiness-associated parallel complaints of cognitive changes (82%), eating disorders (84%); depression (45%); irritability (36%); hypersexuality (18%); and compulsions (11%). Episodes were 8.2 ± 3.3 days in duration. In relapse, diurnal values for blood pressure and heart rate were lower (P < 0.001). In a subgroup (n = 34), cerebrospinal fluid orexin A levels were ∼31% lower in a relapse versus remission (215.7 ± 81.5 versus 319.2 ± 95.92 pg/ml, P < 0.001); in three patients a pattern of lower levels during subsequent relapses was documented.
  
  
  CONCLUSIONS
  There are lower orexin A levels in the symptomatic phase than in remission and a fall and rise in blood pressure and heart rate, suggesting a role for orexin dysregulation in KLS pathophysiology.
authors.affiliation: Binzhou Medical University Hospital, Shandong Province, China.
authors.forename: Jing Yu
authors.initials: JY
authors.lastname: Wang
authors.affiliation: Binzhou Medical University Hospital, Shandong Province, China.|Department of Pulmonary Medicine, Peking University People's Hospital, Beijing, China.
authors.forename: Fang
authors.initials: F
authors.lastname: Han
authors.affiliation: Department of Pulmonary Medicine, Peking University People's Hospital, Beijing, China.
authors.forename: Song X
authors.initials: SX
authors.lastname: Dong
authors.affiliation: Department of Pulmonary Medicine, Peking University People's Hospital, Beijing, China.
authors.forename: Jing
authors.initials: J
authors.lastname: Li
authors.affiliation: Department of Pulmonary Medicine, Peking University People's Hospital, Beijing, China.
authors.forename: Pei
authors.initials: P
authors.lastname: An
authors.affiliation: Department of Pulmonary Medicine, Peking University People's Hospital, Beijing, China.
authors.forename: Xiao Zhe
authors.initials: XZ
authors.lastname: Zhang
authors.affiliation: Department of Pulmonary Medicine, Peking University People's Hospital, Beijing, China.
authors.forename: Yuan
authors.initials: Y
authors.lastname: Chang
authors.affiliation: Department of Pulmonary Medicine, Peking University People's Hospital, Beijing, China.
authors.forename: Long
authors.initials: L
authors.lastname: Zhao
authors.affiliation: Department of Pulmonary Medicine, Peking University People's Hospital, Beijing, China.
authors.forename: Xue Li
authors.initials: XL
authors.lastname: Zhang
authors.affiliation: Department of Pulmonary Medicine, Peking University People's Hospital, Beijing, China.
authors.forename: Ya Nan
authors.initials: YN
authors.lastname: Liu
authors.affiliation: Department of Pulmonary Medicine, Peking University People's Hospital, Beijing, China.
authors.forename: Han
authors.initials: H
authors.lastname: Yan
authors.affiliation: Department of Pulmonary Medicine, Peking University People's Hospital, Beijing, China.
authors.forename: Qing Hua
authors.initials: QH
authors.lastname: Li
authors.affiliation: Department of Pulmonary Medicine, Peking University People's Hospital, Beijing, China.
authors.forename: Yan
authors.initials: Y
authors.lastname: Hu
authors.affiliation: Binzhou Medical University Hospital, Shandong Province, China.
authors.forename: Chang Jun
authors.initials: CJ
authors.lastname: Lv
authors.affiliation: Department of Pulmonary Medicine, Peking University People's Hospital, Beijing, China.
authors.forename: Zhan Cheng
authors.initials: ZC
authors.lastname: Gao
authors.affiliation: Division of Pulmonary, Critical Care and Sleep Medicine, Department of Medicine, Case Western Reserve University, and Cleveland Louis Stokes VA Medical Center, Cleveland, OH.
authors.forename: Kingman P
authors.initials: KP
authors.lastname: Strohl
mesh_terms: D000293:Adolescent; D000328:Adult; D000068476:Beijing; D001794:Blood Pressure; D002648:Child; D005260:Female; D006339:Heart Rate; D006801:Humans; D017593:Kleine-Levin Syndrome / Q000134:cerebrospinal fluid* / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D000068797:Orexins / Q000134:cerebrospinal fluid*; D012008:Recurrence; D055815:Young Adult
chemical_list: D000068797:Orexins
keywords: CSF; hypersomnolence; orexin; syndrome