In vivo evidence of neuronal loss in the hypothalamus of narcoleptic patients

Neurology. 2004 Oct 26;63(8):1513-5. doi: 10.1212/01.wnl.0000142259.94107.4c.

Abstract

A dysfunction of the orexin (hypocretin) system in the hypothalamus has recently been linked to the pathogenesis of narcolepsy. The authors used in vivo proton MR spectroscopy to assess the N-acetylaspartate (NAA) content in the hypothalamus of narcoleptic patients. Hypothalamic NAA/creatine-phosphocreatine was reduced in narcoleptic patients compared with control subjects (p < 0.01). Hypothalamic neuronal loss/damage is a central pathogenetic feature in narcolepsy.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aspartic Acid / analogs & derivatives
  • Aspartic Acid / analysis
  • Aspartic Acid / deficiency
  • Aspartic Acid / metabolism
  • Atrophy / diagnosis*
  • Atrophy / metabolism
  • Atrophy / physiopathology
  • Cataplexy / etiology
  • Cataplexy / pathology
  • Cataplexy / physiopathology
  • Creatine / metabolism
  • Female
  • Humans
  • Hypothalamus / metabolism
  • Hypothalamus / pathology*
  • Hypothalamus / physiopathology
  • Intracellular Signaling Peptides and Proteins / deficiency
  • Magnetic Resonance Imaging
  • Magnetic Resonance Spectroscopy
  • Male
  • Middle Aged
  • Narcolepsy / diagnosis*
  • Narcolepsy / metabolism
  • Narcolepsy / physiopathology
  • Nerve Degeneration / diagnosis*
  • Nerve Degeneration / metabolism
  • Nerve Degeneration / physiopathology
  • Neurons / metabolism
  • Neurons / pathology*
  • Neuropeptides / deficiency
  • Orexins
  • Polysomnography

Substances

  • Intracellular Signaling Peptides and Proteins
  • Neuropeptides
  • Orexins
  • Aspartic Acid
  • N-acetylaspartate
  • Creatine