Hypocretin (orexin) levels in cerebrospinal fluid of patients with narcolepsy: Relationship to cataplexy and HLA DQB1*0602 Status

Lois Elaine Krahn, V. Shane Pankratz, Lawrence Oliver, Bradley F Boeve, Michael H. Silber

Research output: Contribution to journalArticle

111 Citations (Scopus)

Abstract

Study Objectives: Hypocretin (orexin) deficiency (< 40 pg/ml) is highly associated with narcolepsy with cataplexy (89.5%) and more specifically with patients with cataplexy who are HLA DQB1*0602 positive (95.7%). The relationship between hypocretin-1 levels and narcolepsy without cataplexy or the DQB1*0602 allele is less clear. Methods/Design: This study compared cerebrospinal (CSF) hypocretin 1 in 13 patients with HLA DQB1*0602 allele and cataplexy to 4 HLA negative patients with cataplexy, 3 HLA positive patients without cataplexy, and 6 HLA negative patients without cataplexy, plus 15 neurologic controls. Setting: Data were collected at a sleep disorders center. Patients/Participants: Twenty-six patients with narcolepsy, with and without HLA DQB1*0602 and with and without cataplexy, as well as 15 neurologic controls. Interventions: N/A Measurement & Results: Using analysis of variance techniques, statistically significant differences were found between the CSF hypocretin-1 levels in HLA positive patients and all other groups (P<0.01). Although the sample size was small, subjects with the DQB1*0602 allele without cataplexy had lower hypocretin-1 levels than did other groups (other than the HLA and cataplexy positive group). Hypocretin-1 levels were not associated with age at diagnosis, age at lumbar puncture, body mass index at time of diagnosis, or body mass index at time of lumbar puncture. Conclusion: This data confirms the previous finding that undetectable hypocretin-1 levels are highly specific for HLA positive narcolepsy with cataplexy. The data suggests that the pathophysiology and, by extension, etiology of this disorder are distinctly different from the other conditions studied. The relationship of the DQB1*0602 allele and reduced hypocretin-1 levels needs further study.

Original languageEnglish (US)
Pages (from-to)733-736
Number of pages4
JournalSleep
Volume25
Issue number7
StatePublished - Nov 1 2002

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Cataplexy
Narcolepsy
Cerebrospinal Fluid
Alleles
Macrophage Colony-Stimulating Factor
Spinal Puncture
Nervous System
Body Mass Index
HLA-DQB1 antigen
Orexins
Sample Size
Analysis of Variance

Keywords

  • Cataplexy
  • Cerebrospinal
  • Hypocretin
  • Narcolepsy
  • Orexin

ASJC Scopus subject areas

  • Physiology

Cite this

Hypocretin (orexin) levels in cerebrospinal fluid of patients with narcolepsy : Relationship to cataplexy and HLA DQB1*0602 Status. / Krahn, Lois Elaine; Pankratz, V. Shane; Oliver, Lawrence; Boeve, Bradley F; Silber, Michael H.

In: Sleep, Vol. 25, No. 7, 01.11.2002, p. 733-736.

Research output: Contribution to journalArticle

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abstract = "Study Objectives: Hypocretin (orexin) deficiency (< 40 pg/ml) is highly associated with narcolepsy with cataplexy (89.5{\%}) and more specifically with patients with cataplexy who are HLA DQB1*0602 positive (95.7{\%}). The relationship between hypocretin-1 levels and narcolepsy without cataplexy or the DQB1*0602 allele is less clear. Methods/Design: This study compared cerebrospinal (CSF) hypocretin 1 in 13 patients with HLA DQB1*0602 allele and cataplexy to 4 HLA negative patients with cataplexy, 3 HLA positive patients without cataplexy, and 6 HLA negative patients without cataplexy, plus 15 neurologic controls. Setting: Data were collected at a sleep disorders center. Patients/Participants: Twenty-six patients with narcolepsy, with and without HLA DQB1*0602 and with and without cataplexy, as well as 15 neurologic controls. Interventions: N/A Measurement & Results: Using analysis of variance techniques, statistically significant differences were found between the CSF hypocretin-1 levels in HLA positive patients and all other groups (P<0.01). Although the sample size was small, subjects with the DQB1*0602 allele without cataplexy had lower hypocretin-1 levels than did other groups (other than the HLA and cataplexy positive group). Hypocretin-1 levels were not associated with age at diagnosis, age at lumbar puncture, body mass index at time of diagnosis, or body mass index at time of lumbar puncture. Conclusion: This data confirms the previous finding that undetectable hypocretin-1 levels are highly specific for HLA positive narcolepsy with cataplexy. The data suggests that the pathophysiology and, by extension, etiology of this disorder are distinctly different from the other conditions studied. The relationship of the DQB1*0602 allele and reduced hypocretin-1 levels needs further study.",
keywords = "Cataplexy, Cerebrospinal, Hypocretin, Narcolepsy, Orexin",
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AU - Oliver, Lawrence

AU - Boeve, Bradley F

AU - Silber, Michael H.

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N2 - Study Objectives: Hypocretin (orexin) deficiency (< 40 pg/ml) is highly associated with narcolepsy with cataplexy (89.5%) and more specifically with patients with cataplexy who are HLA DQB1*0602 positive (95.7%). The relationship between hypocretin-1 levels and narcolepsy without cataplexy or the DQB1*0602 allele is less clear. Methods/Design: This study compared cerebrospinal (CSF) hypocretin 1 in 13 patients with HLA DQB1*0602 allele and cataplexy to 4 HLA negative patients with cataplexy, 3 HLA positive patients without cataplexy, and 6 HLA negative patients without cataplexy, plus 15 neurologic controls. Setting: Data were collected at a sleep disorders center. Patients/Participants: Twenty-six patients with narcolepsy, with and without HLA DQB1*0602 and with and without cataplexy, as well as 15 neurologic controls. Interventions: N/A Measurement & Results: Using analysis of variance techniques, statistically significant differences were found between the CSF hypocretin-1 levels in HLA positive patients and all other groups (P<0.01). Although the sample size was small, subjects with the DQB1*0602 allele without cataplexy had lower hypocretin-1 levels than did other groups (other than the HLA and cataplexy positive group). Hypocretin-1 levels were not associated with age at diagnosis, age at lumbar puncture, body mass index at time of diagnosis, or body mass index at time of lumbar puncture. Conclusion: This data confirms the previous finding that undetectable hypocretin-1 levels are highly specific for HLA positive narcolepsy with cataplexy. The data suggests that the pathophysiology and, by extension, etiology of this disorder are distinctly different from the other conditions studied. The relationship of the DQB1*0602 allele and reduced hypocretin-1 levels needs further study.

AB - Study Objectives: Hypocretin (orexin) deficiency (< 40 pg/ml) is highly associated with narcolepsy with cataplexy (89.5%) and more specifically with patients with cataplexy who are HLA DQB1*0602 positive (95.7%). The relationship between hypocretin-1 levels and narcolepsy without cataplexy or the DQB1*0602 allele is less clear. Methods/Design: This study compared cerebrospinal (CSF) hypocretin 1 in 13 patients with HLA DQB1*0602 allele and cataplexy to 4 HLA negative patients with cataplexy, 3 HLA positive patients without cataplexy, and 6 HLA negative patients without cataplexy, plus 15 neurologic controls. Setting: Data were collected at a sleep disorders center. Patients/Participants: Twenty-six patients with narcolepsy, with and without HLA DQB1*0602 and with and without cataplexy, as well as 15 neurologic controls. Interventions: N/A Measurement & Results: Using analysis of variance techniques, statistically significant differences were found between the CSF hypocretin-1 levels in HLA positive patients and all other groups (P<0.01). Although the sample size was small, subjects with the DQB1*0602 allele without cataplexy had lower hypocretin-1 levels than did other groups (other than the HLA and cataplexy positive group). Hypocretin-1 levels were not associated with age at diagnosis, age at lumbar puncture, body mass index at time of diagnosis, or body mass index at time of lumbar puncture. Conclusion: This data confirms the previous finding that undetectable hypocretin-1 levels are highly specific for HLA positive narcolepsy with cataplexy. The data suggests that the pathophysiology and, by extension, etiology of this disorder are distinctly different from the other conditions studied. The relationship of the DQB1*0602 allele and reduced hypocretin-1 levels needs further study.

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