The onset of narcolepsy-cataplexy is typically in adolescence and not at birth, suggesting that the disease may require environmental influence to develop. Currently, it is believed that narcolepsy results from autoimmune insult to the CNS because it is associated with HLA (major histocompatibility complex) DQB1*0602 and DQ1A1*0102.17,18 Concentrations of hypocretin (a wake-promoting neuropeptide) in the cerebrospinal fluid (CSF) of narcolepsy patients are reduced significantly, suggesting that the autoimmune attack is against hypocretin-producing cells in the hypothalamus.19 Intact hypocretin neurons normally stimulate arousal and wake-promoting neurons to stimulate cortical activation and behavioral arousal.

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