Hypocretin (orexin) deficiency predicts severe objective excessive daytime sleepiness in narcolepsy with cataplexy

J Neurol Neurosurg Psychiatry. 2006 Mar;77(3):402-4. doi: 10.1136/jnnp.2005.067207.

Abstract

Cerebrospinal fluid (CSF) hypocretin-1 deficiency is associated with definite ("clear cut") cataplexy in patients with narcolepsy. The relationship between CSF hypocretin-1 levels and other narcoleptic symptoms (including excessive daytime sleepiness, EDS) is not properly understood. In a consecutive series of 18 subjects with narcolepsy and definite cataplexy, patients with undetectable CSF hypocretin-1 (n = 12) were found to have significantly lower mean sleep latencies (p = 0.045) and a higher frequency of sleep onset REM periods (SOREMPs, p = 0.025) on multiple sleep latency test than patients (n = 6) with detectable levels. Conversely, Epworth sleepiness scale scores, the frequency of hallucinations/sleep paralysis, and the frequency and severity of cataplexy were similar in both groups. These results suggest that hypocretin deficiency identifies a homogenous group of patients with narcolepsy characterised by the presence of definite cataplexy, severe EDS, and frequent SOREMPs.

MeSH terms

  • Adolescent
  • Adult
  • Cataplexy / cerebrospinal fluid*
  • Cataplexy / diagnosis*
  • Disorders of Excessive Somnolence / cerebrospinal fluid*
  • Disorders of Excessive Somnolence / diagnosis
  • Female
  • Humans
  • Intracellular Signaling Peptides and Proteins / deficiency*
  • Male
  • Middle Aged
  • Narcolepsy / cerebrospinal fluid*
  • Narcolepsy / diagnosis
  • Neuropeptides / deficiency*
  • Orexins
  • Polysomnography
  • Predictive Value of Tests
  • Prognosis
  • Sleep, REM / physiology

Substances

  • HCRT protein, human
  • Intracellular Signaling Peptides and Proteins
  • Neuropeptides
  • Orexins