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

TY - JOUR

T1 - Narcolepsy with hypocretin/orexin deficiency, infections and autoimmunity of the brain

AU - Kornum, Birgitte Rahbek

AU - Faraco, Juliette

AU - Mignot, Emmanuel

N1 - Copyright © 2011 Elsevier Ltd. All rights reserved.

PY - 2011/12

Y1 - 2011/12

N2 - 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.

AB - 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.

KW - Autoimmune Diseases/immunology

KW - Brain/immunology

KW - Humans

KW - Intracellular Signaling Peptides and Proteins/deficiency

KW - Narcolepsy/immunology

KW - Neuropeptides/deficiency

KW - Orexins

U2 - 10.1016/j.conb.2011.09.003

DO - 10.1016/j.conb.2011.09.003

M3 - Review

C2 - 21963829

SN - 0959-4388

VL - 21

SP - 897

EP - 903

JO - Current Opinion in Neurobiology

JF - Current Opinion in Neurobiology

IS - 6

ER -