Narcolepsy with hypocretin/orexin deficiency, infections and autoimmunity of the brain

Birgitte Rahbek Kornum, Juliette Faraco, Emmanuel Mignot

114 Citations (Scopus)

Abstract

The loss of hypothalamic hypocretin/orexin (hcrt) producing neurons causes narcolepsy with cataplexy. An autoimmune basis for the disease has long been suspected and recent results have greatly strengthened this hypothesis. Narcolepsy with hcrt deficiency is now known to be associated with a Human Leukocyte Antigen (HLA) and T-cell receptor (TCR) polymorphisms, suggesting that an autoimmune process targets a single peptide unique to hcrt-cells via specific HLA-peptide-TCR interactions. Recent data have shown a robust seasonality of disease onset in children and associations with Streptococcus Pyogenes, and influenza A H1N1-infection and H1N1-vaccination, pointing towards processes such as molecular mimicry or bystander activation as crucial for disease development. We speculate that upper airway infections may be common precipitants of a whole host of CNS autoimmune complications including narcolepsy.

Original languageEnglish
JournalCurrent Opinion in Neurobiology
Volume21
Issue number6
Pages (from-to)897-903
Number of pages7
ISSN0959-4388
DOIs
Publication statusPublished - Dec 2011
Externally publishedYes

Keywords

  • Autoimmune Diseases
  • Brain
  • Humans
  • Intracellular Signaling Peptides and Proteins
  • Narcolepsy
  • Neuropeptides

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