Format

Send to

Choose Destination
Curr Opin Neurobiol. 2011 Dec;21(6):897-903. doi: 10.1016/j.conb.2011.09.003. Epub 2011 Sep 30.

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

Author information

1
Stanford Center for Sleep Sciences and Medicine, Stanford University School of Medicine, Palo Alto, CA 94304, USA.

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.

PMID:
21963829
DOI:
10.1016/j.conb.2011.09.003
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center