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Narcolepsy with Cataplexy: Hypocretin and Immunological Aspects

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Neuroimmunology of Sleep

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Abstract

Narcolepsy is a disabling sleep disorder characterized by severe excessive daytime sleepiness and abnormal rapid eye movement (REM) sleep manifestations including cataplexy, sleep paralysis, hypnagogic hallucinations, and sleep onset REM periods (American Academy of Sleep Medicine 2005; Dauvilliers, Billiard, and Montplaisir 2003b; Scammell 2003). Recent advances in pathophysiology demonstrated that narcolepsy is caused by the loss of hypothalamic neurons producing hypocretin (Mignot et al. 2002; Dauvilliers et al. 2003a; Peyron et al. 2000; Thannickal et al. 2000; Crocker et al. 2005; Blouin, Thannickal, Worley, Baraban, Reti, and Siegel 2005). Epidemiological data with a young and bimodal age at onset, frequent triggering factors and a tight HLA DQB1*0602 association, suggest an autoimmune hypothesis (Mignot, Tafti, Dement, and Grumet 1995; Carlander, Dauvilliers, and Billiard 2001; Chabas, Taheri, Renier, and Mignot 2003). Acting on a specific genetic background, an autoimmune process targeting hypocretin neurons, in response to yet unknown environmental factors, is the most probable hypothesis. The treatment of narcolepsy has evolved significantly over the last few years, but current therapies are only symptom-based. Hypocretin-based therapies and immune-based therapies are part of the research projects for the development of new treatments in narcolepsy (Dauvilliers and Tafti 2006).

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Authors
  1. Yves Dauvilliers
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Editors and Affiliations

  1. Mount Sinai School of Medicine, New York, NY, USA

    S. R. Pandi-Perumal

  2. University of Buenow Aires, Buenos Aires, Argentina

    Daniel P. Cardinali

  3. Athens University Medical School, Athens, Greece

    George P. Chrousos

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Dauvilliers, Y. (2007). Narcolepsy with Cataplexy: Hypocretin and Immunological Aspects. In: Pandi-Perumal, S., Cardinali, D.P., Chrousos, G.P. (eds) Neuroimmunology of Sleep. Springer, Boston, MA. https://doi.org/10.1007/978-0-387-69146-6_20

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