SLEEP AND RESPIRATORY NEUROBIOLOGY: Edited by Lee K. Brown and Adrian J. WilliamsAutoimmunity in narcolepsyBonvalet, Melodiea; Ollila, Hanna M.a,b,c; Ambati, Adityaa; Mignot, EmmanuelaAuthor Information aDepartment of Psychiatry and Behavioral Sciences, Center for Sleep Sciences, Stanford University School of Medicine, Palo Alto, California, USA bNational Institute for Health and Welfare, Public Genomics Unit cInstitute for Molecular Medicine FIMM, University of Helsinki, Helsinki, Finland Correspondence to Emmanuel Mignot, Stanford University Center for Sleep Sciences, 3165 Porter Drive, Palo Alto, CA 94304, USA. Tel: +1 650 725 6517; fax: +1 650 725 4913; e-mail: [email protected] Current Opinion in Pulmonary Medicine: November 2017 - Volume 23 - Issue 6 - p 522-529 doi: 10.1097/MCP.0000000000000426 Buy Metrics Abstract Purpose of review Summarize the recent findings in narcolepsy focusing on the environmental and genetic risk factors in disease development. Recent findings Both genetic and epidemiological evidence point towards an autoimmune mechanism in the destruction of orexin/hypocretin neurons. Recent studies suggest both humoral and cellular immune responses in the disease development. Summary Narcolepsy is a severe sleep disorder, in which neurons producing orexin/hypocretin in the hypothalamus are destroyed. The core symptoms of narcolepsy are debilitating, extreme sleepiness, cataplexy, and abnormalities in the structure of sleep. Both genetic and epidemiological evidence point towards an autoimmune mechanism in the destruction of orexin/hypocretin neurons. Importantly, the highest environmental risk is seen with influenza-A infection and immunization. However, how the cells are destroyed is currently unknown. In this review we summarize the disease symptoms, and focus on the immunological findings in narcolepsy. We also discuss the environmental and genetic risk factors as well as propose a model for disease development. Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.