title: Report of a case of immunosuppression with prednisone in an 8-year-old boy with an acute onset of hypocretin-deficiency narcolepsy.
abstract: OBJECTIVE
  To explore whether acute destruction of hypocretin cells in a patient with narcolepsy could be detected and if the course of the disease could be reversed or altered by the use of prednisone for immunosuppression.
  
  
  METHODS
  Case report.
  
  
  METHODS
  A sleep-clinic population in a tertiary-care hospital.
  
  
  METHODS
  An 8-year-old boy with a very acute recent (< 2 month) onset of sleepiness.
  
  
  METHODS
  Sleep studies; fluid-attenuated inversion recovery and gadolinium magnetic resonance imaging studies with a focus on the hypothalamus; examinations of cerebrospinal fluid for cytology, protein, and hypocretin-1 levels; and HLA typing were performed.
  
  
  METHODS
  A 3-week regimen of 1 mg x kg(-1) x day(-1) of prednisone was administered in an attempt to modify the course of the disease.
  
  
  CONCLUSIONS
  Sleep evaluations were consistent with a diagnosis of narcolepsy. Hypocretin-1 was absent in the cerebrospinal fluid, and HLA-DQB1*0602 was present. All other results were within normal limits, and prednisone did not have any noticeable effects. Clinical manifestation of narcolepsy might occur when the hypocretin cell damage is too advanced to be reversible.
authors.affiliation: Center For Narcolepsy, Department of Pediatrics, Stanford University School of Medicine, Stanford, Calif, USA.
authors.forename: Marketa
authors.initials: M
authors.lastname: Hecht
authors.forename: Ling
authors.initials: L
authors.lastname: Lin
authors.forename: Clete A
authors.initials: CA
authors.lastname: Kushida
authors.forename: Dale T
authors.initials: DT
authors.lastname: Umetsu
authors.forename: Shahrad
authors.initials: S
authors.lastname: Taheri
authors.forename: Mali
authors.initials: M
authors.lastname: Einen
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Mignot
mesh_terms: D000208:Acute Disease; D001559:Benzhydryl Compounds / Q000627:therapeutic use; D002352:Carrier Proteins / Q000134:cerebrospinal fluid; D000697:Central Nervous System Stimulants / Q000627:therapeutic use; D002648:Child; D003511:Cyclohexanols / Q000627:therapeutic use; D006680:HLA Antigens / Q000276:immunology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D047908:Intracellular Signaling Peptides and Proteins; D008297:Male; D000077408:Modafinil; D009290:Narcolepsy / Q000175:diagnosis / Q000188:drug therapy* / Q000378:metabolism*; D009479:Neuropeptides / Q000134:cerebrospinal fluid / Q000172:deficiency*; D000068797:Orexins; D017286:Polysomnography; D011241:Prednisone / Q000627:therapeutic use*; D017367:Selective Serotonin Reuptake Inhibitors / Q000627:therapeutic use; D000069470:Venlafaxine Hydrochloride
chemical_list: D001559:Benzhydryl Compounds; D002352:Carrier Proteins; D000697:Central Nervous System Stimulants; D003511:Cyclohexanols; D006680:HLA Antigens; D007166:Immunosuppressive Agents; D047908:Intracellular Signaling Peptides and Proteins; D000077408:Modafinil; D009479:Neuropeptides; D000068797:Orexins; D011241:Prednisone; D017367:Selective Serotonin Reuptake Inhibitors; D000069470:Venlafaxine Hydrochloride; C000596229:HCRT protein, human