Epidemiology of narcolepsy

The prevalence of narcolepsy is usually presented at about 50/100,000. There are, however, marked differences of about 2,500-fold between the lowest and the highest reported prevalence. This discrepancy is at least partly explained by differences in the study populations and methods. There are, however, no earlier population-based epidemiological studies with polygraphically confirmed diagnoses. We studied the occurrence of symptoms resembling the two main manifestations of narcolepsy, i.e. abnormal sleep tendency and emotion-associated muscular weakness, in an adult twin cohort (n = 16,179) with a questionnaire. A total of 3.2% met the minimal diagnostic criteria of the International Classification of Sleep Disorders for narcolepsy. Eleven questionnaire items assessing the main manifestations of narcolepsy formed a measure called the Ullanlinna Narcolepsy Scale (UNS), which has been validated. The UNS score was calculated for 11,354 subjects. Those (n = 75) having a UNS score equal or higher than the lowest value in a narcolepsy patient group were studied. Thirty-one of them (fulfilling also the minimal diagnostic criteria) were interviewed, and those suspected of having narcolepsy (n = 5) were evaluated in the sleep laboratory. In three subjects the narcolepsy diagnosis was verified (all dizygotic, nonfamilial and human leukocyte antigen DR2/DQB-0602 positive), giving a prevalence of 0.026% in the adult Finnish (Caucasian) population.