title: CSF hypocretin-1 levels and clinical profiles in narcolepsy and idiopathic CNS hypersomnia in Norway.
abstract: OBJECTIVE
  To evaluate the relationship between CSF hypocretin-1 levels and clinical profiles in narcolepsy and CNS hypersomnia in Norwegian patients.
  
  
  METHODS
  CSF hypocretin-1 was measured by a sensitive radioimmunoassay in 47 patients with narcolepsy with cataplexy, 7 with narcolepsy without cataplexy, 10 with idiopathic CNS hypersomnia, and a control group.
  
  
  RESULTS
  Low hypocretin-1 values were found in 72% of the HLA DQB1*0602 positive patients with narcolepsy and cataplexy. Patients with low CSF hypocretin-1 levels reported more extensive muscular involvement during cataplectic attacks than patients with normal levels. Hypnagogic hallucinations and sleep paralysis occurred more frequently in patients with cataplexy than in the other patient groups, but with no correlation to hypocretin-1 levels.
  
  
  CONCLUSIONS
  About three quarters of the HLA DQB1*0602 positive patients with narcolepsy and cataplexy had low CSF hypocretin-1 values, and appear to form a distinct clinical entity. Narcolepsy without cataplexy could not be distinguished from idiopathic CNS hypersomnia by clinical symptoms or biochemical findings.
authors: Department of Neurology, Ullevål University Hospital, Oslo, Norway. msheier@online.no
authors: Mona Skard
authors: MS
authors: Heier
authors: Tatiana
authors: T
authors: Evsiukova
authors: Steinar
authors: S
authors: Vilming
authors: Michaela D
authors: MD
authors: Gjerstad
authors: Harald
authors: H
authors: Schrader
authors: Kaare
authors: K
authors: Gautvik
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002385:Cataplexy / Q000134:cerebrospinal fluid* / Q000175:diagnosis / Q000235:genetics; D005260:Female; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006683:HLA-DQ Antigens / Q000235:genetics; D059866:HLA-DQ beta-Chains; D006212:Hallucinations / Q000134:cerebrospinal fluid / Q000175:diagnosis / Q000235:genetics; D006801:Humans; D020177:Idiopathic Hypersomnia / Q000134:cerebrospinal fluid* / Q000175:diagnosis / Q000235:genetics; D047908:Intracellular Signaling Peptides and Proteins / Q000134:cerebrospinal fluid* / Q000235:genetics; D008297:Male; D008562:Membrane Glycoproteins / Q000235:genetics; D009290:Narcolepsy / Q000134:cerebrospinal fluid* / Q000175:diagnosis / Q000235:genetics; D009479:Neuropeptides / Q000134:cerebrospinal fluid* / Q000235:genetics; D000068797:Orexins; D011863:Radioimmunoassay; D012016:Reference Values; D012307:Risk Factors; D020188:Sleep Paralysis / Q000134:cerebrospinal fluid / Q000175:diagnosis / Q000235:genetics
chemical_list: C000596229:HCRT protein, human; D006683:HLA-DQ Antigens; D059866:HLA-DQ beta-Chains; C069051:HLA-DQB1 antigen; D047908:Intracellular Signaling Peptides and Proteins; D008562:Membrane Glycoproteins; D009479:Neuropeptides; D000068797:Orexins
references: Proc Natl Acad Sci U S A. 1996 Aug 6;93(16):8733-8
references: 8710940
references: Neurology. 1998 Feb;50(2 Suppl 1):S2-7
references: 9484416
references: J Neurol Neurosurg Psychiatry. 2003 Dec;74(12):1667-73
references: 14638887
references: Sleep. 2002 Nov 1;25(7):733-6
references: 12405608
references: J Sleep Res. 2005 Dec;14(4):437-45
references: 16364145
references: Sleep. 1994 Dec;17(8 Suppl):S7-12
references: 7701204
references: Neurology. 2001 Nov 27;57(10):1893-5
references: 11723284
references: Neurology. 2001 Nov 27;57(10):1896-9
references: 11723285
references: Neurology. 2002 Jun 25;58(12):1826-33
references: 12084885
references: Cell. 1999 Aug 20;98(4):437-51
references: 10481909
references: Sleep Med. 2001 Sep;2(5):451-3
references: 14592396
references: Ann Neurol. 1994 Jun;35(6):709-16
references: 8210228
references: J Neurosci. 1998 Dec 1;18(23):9996-10015
references: 9822755
references: Sleep. 2002 Feb 1;25(1):27-35
references: 11833858
references: Neuron. 2000 Sep;27(3):469-74
references: 11055430
references: J Comp Neurol. 1998 Dec 28;402(4):460-74
references: 9862321
references: Neurology. 2001 Dec 26;57(12):2253-8
references: 11756606
references: Sleep. 1997 Aug;20(8):620-9
references: 9351129
references: Arch Neurol. 2002 Oct;59(10):1553-62
references: 12374492
references: Cell. 1999 Aug 6;98(3):365-76
references: 10458611
references: Sleep Med. 2003 Jan;4(1):7-12
references: 14592354
references: Proc Natl Acad Sci U S A. 1998 Jan 6;95(1):322-7
references: 9419374
references: Sleep. 1997 Nov;20(11):1012-20
references: 9456467
references: J Sleep Res. 2004 Dec;13(4):395-406
references: 15560774
references: Lancet. 2000 Jan 1;355(9197):39-40
references: 10615891
references: J R Soc Med. 2002 May;95(5):227-30
references: 11983761
references: FEBS J. 2005 Nov;272(22):5675-88
references: 16279933
references: Sleep. 1997 Nov;20(11):1021-6
references: 9456468