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

Birgitte Rahbek Kornum, Juliette Faraco, Emmanuel Mignot

    113 Citationer (Scopus)


    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.

    TidsskriftCurrent Opinion in Neurobiology
    Udgave nummer6
    Sider (fra-til)897-903
    Antal sider7
    StatusUdgivet - dec. 2011


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