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Sleep Disorders
Volume 2014 (2014), Article ID 792687, 6 pages
http://dx.doi.org/10.1155/2014/792687
Review Article

Narcolepsy as an Immune-Mediated Disease

1Stanford Center for Sleep Sciences and Medicine, Stanford University School of Medicine, 3165 Porter Drive, Palo Alto, CA 94304, USA
2Instituto de Ciencias de la Salud, Departamento de Biomedicina, Universidad Veracruzana, Industrial-Animas, 91190 Xalapa, VER, Mexico
3Laboratory of Sleep Biology, Department of Biomedicine, Institute of Health Sciences, Veracruzana University, Avenida Luis Castelazo Ayala s/n, Industrial-Animas, 91190 Xalapa, VER, Mexico

Received 30 June 2013; Revised 30 September 2013; Accepted 19 October 2013; Published 14 January 2014

Academic Editor: Michel M. Billiard

Copyright © 2014 Alberto K. De la Herrán-Arita and Fabio García-García. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Narcolepsy is a neurological disorder characterized by excessive daytime sleepiness, cataplexy, hypnagonic hallucinations, sleep paralysis, and disturbed nocturnal sleep patterns. This disease is secondary to the specific loss of hypothalamic hypocretin (orexin)-producing neurons in the lateral hypothalamus. An autoimmune basis for the disease has long been suspected based on its strong association with the genetic marker DQB1*06:02, and current studies greatly support this hypothesis. Narcolepsy with hypocretin deficiency is associated with human leukocyte antigen (HLA) and T cell receptor (TCR) polymorphisms, suggesting that an autoimmune process targets a peptide unique to hypocretin-producing neurons via specific HLA-peptide-TCR interactions. This concept has gained a lot of notoriety after the increase of childhood narcolepsy in 2010 following the 2009 H1N1 pandemic (pH1N1) in China and vaccination with Pandemrix, an adjuvanted H1N1 vaccine that was used in Scandinavia. The surge of narcolepsy cases subsequent to influenza A H1N1 infection and H1N1 vaccination suggests that processes such as molecular mimicry or bystander activation might be crucial for disease development.