Narcolepsy Presentation in Diverse Populations: an Update
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SLEEP AND HEALTH DISPARITIES (A SHELTON, SECTION EDITOR)
Narcolepsy Presentation in Diverse Populations: an Update Karen Spruyt 1 Accepted: 29 October 2020 / Published online: 25 November 2020 # Springer Nature Switzerland AG 2020
Abstract Purpose of Review We performed a literature search to generate incidence and prevalence rates of narcolepsy in diverse populations based on current available data. Recent Findings With an onset in childhood, narcolepsy often has a delayed diagnosis due to symptoms of excessive daytime sleepiness not being recognized or being misdiagnosed. Clinical, electrophysiological, and biological tests are needed in order to diagnose narcolepsy. At the same time, the discovery of the link with the immunoregulatory human leukocyte antigen complex and the adverse events in relation to the H1N1 pandemic vaccines have shuffled the epidemiological numbers. Summary In this meta-review, we pooled incidence rates and prevalence rates reported in 30 countries or from 209 sets of data. Findings are reported per age, continent, and proxy race/ethnicity as well as period (i.e., before/after the pandemic). This metareview showed that narcolepsy occurs in 0.87–1.21 of the world population, with specifically NT1 being investigated. Its pooled incidence rate in vaccinated samples is 1.58. There is furthermore an underreporting of narcolepsy in ethnic/race and gender minorities, of childhood narcolepsy type 2 and potential comorbid conditions masking the clinical complaints and hence timely diagnosis. Keywords Narcolepsy . Excessive daytime somnolence . Prevalence . Incidence
Introduction Narcolepsy is a chronic sleep disorder characterized by excessive daytime sleepiness with or without cataplexy, or episodes of muscle weakness triggered by strong emotions: narcolepsy type 1 (NT1) and narcolepsy type 2 (NT2), respectively [1]. Both genetic and epidemiological evidence suggest an autoimmune mechanism in the destruction, or a highly specific loss, of orexin/ hypocretin neurons, while influenza A infection and immunization have been proposed as the highest environmental risk factors [2•, 3]. Overnight polysomnography and the multiple sleep latency test (MSLT) reveal short sleep latencies and rapid eye movement (REM) periods characterizing the sleep This article is part of the Topical Collection on Sleep and Health Disparities * Karen Spruyt [email protected]; [email protected] 1
School of Medicine, INSERM, University Claude Bernard, Lyon, France
architecture of individuals with narcolepsy [1]. Human leukocyte antigen (HLA) typing has been suggested as a useful test to screen familial risk [4••]; however, susceptibility for a number of neurodegenerative diseases, for example, Alzheimer disease, equally associates to this immunoregulatory complex. To date, the diagnosis of narcolepsy is still secured by clinical, electrophysiological, and biological evaluations often leading to a delayed diagnosis, e.g., 8.7 to 22.1 years [5], at risk of misdiagnosis [6]. In 2010, Sweden and Finland flagged adve
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