title: Preliminary results on CSF biomarkers for hypothalamic dysfunction in Kleine-Levin syndrome.
abstract: OBJECTIVE
  To measure CSF biomarkers of hypothalamic dysfunction in patients with typical Kleine-Levin syndrome (KLS) during symptomatic and asymptomatic periods.
  
  
  METHODS
  Two patients with typical KLS were admitted during symptomatic and asymptomatic periods to a research Sleep Disorders Center. Cerebrospinalfluid (CSF) hypocretin-1, histamine (HA), and its major metabolite tele-methylhistamine (t-MHA) levels were measured in two KLS patients in and out of episode.
  
  
  RESULTS
  CSF biomarkers of hypothalamic dysfunction measured in two KLS patients in and out of episode revealed low hypocretin levels (within the narcolepsy-cataplexy range) during a hypersomnia episode in the more severe patient, and a 42% decrease (although within normal range) in the second patient. CSF HA and t-MHA measurements in and out of episode revealed a two-fold in-episode decrease in HA in the more severe patient, with no significant change for the second patient, nor for t-MHA levels.
  
  
  CONCLUSIONS
  We reported reversible changes in CSF hypothalamic biomarkers in a typical patient with KLS that reinforces the hypothesis that in some patients KLS episodes may be caused by recurrent functional alterations of the hypothalamus.
authors.affiliation: Service de Neurologie, Hôpital Gui-de-Chauliac, CHU, Montpellier, France; Centre de Référence Nationale Maladie Rare, Narcolepsie et Hypersomnie Idiopathique, Montpellier, France; Inserm U1061, Montpellier, France.
authors.forename: Régis
authors.initials: R
authors.lastname: Lopez
authors.affiliation: Service de Neurologie, Hôpital Gui-de-Chauliac, CHU, Montpellier, France; Centre de Référence Nationale Maladie Rare, Narcolepsie et Hypersomnie Idiopathique, Montpellier, France.
authors.forename: Lucie
authors.initials: L
authors.lastname: Barateau
authors.affiliation: Service de Neurologie, Hôpital Gui-de-Chauliac, CHU, Montpellier, France; Centre de Référence Nationale Maladie Rare, Narcolepsie et Hypersomnie Idiopathique, Montpellier, France.
authors.forename: Sofiene
authors.initials: S
authors.lastname: Chenini
authors.affiliation: Service de Neurologie, Hôpital Gui-de-Chauliac, CHU, Montpellier, France; Centre de Référence Nationale Maladie Rare, Narcolepsie et Hypersomnie Idiopathique, Montpellier, France; Inserm U1061, Montpellier, France. Electronic address: ydauvilliers@yahoo.fr.
authors.forename: Yves
authors.initials: Y
authors.lastname: Dauvilliers
mesh_terms: D000293:Adolescent; D015415:Biomarkers / Q000134:cerebrospinal fluid; D006801:Humans; D007027:Hypothalamic Diseases / Q000134:cerebrospinal fluid* / Q000175:diagnosis* / Q000209:etiology; D017593:Kleine-Levin Syndrome / Q000134:cerebrospinal fluid* / Q000150:complications*; D008297:Male; D008761:Methylhistamines / Q000134:cerebrospinal fluid*; D000068797:Orexins / Q000134:cerebrospinal fluid*
chemical_list: D015415:Biomarkers; D008761:Methylhistamines; D000068797:Orexins; C020404:tele-methylhistamine
keywords: Histamine; Hypocretin; Hypothalamus; Kleine–Levin syndrome; Telemethyl histamin