21706_NarcolepsyInChildren

In this symposium, experts will address best practices in the diagnosis and treatment of narcolepsy, and explore hypersomnia mimics of narcolepsy in children.

The diagnosis of narcolepsy in children is fraught with difficulties, complicated, in part, by a dearth of clinical data. When presenting in the preschool age, the diagnosis is often confounded by the inability of young children to describe the features of cataplexy, hallucinations, and sleep paralysis. Additionally, the prevalence of daytime napping in normal children in this age group may make it more difficult to identify Excessive Daytime Sleepiness (EDS). The absence of normative data for the Multiple Sleep Latency Test (MSLT) in pre-pubertal children further complicates diagnosis. In school-age children with narcolepsy, the re-emergence of daytime napping may be seen; however, EDS may also present as irritability, inattentiveness, and decreased school performance, which may be misdiagnosed as attention deficit hyperactivity disorder (ADHD). Hallucinations may also be misinterpreted as signs of a primary psychiatric disorder, and cataplexy may also be confused with a variety of neurologic conditions including epileptic drop attacks. Furthermore, clinicians must be able to differentiate hypersomnia mimics of narcolepsy in children such as delayed sleep phase syndrome in teenagers, Kleine-Levin Syndrome, autoimmune encephalitis, sedating medications, and traumatic brain injury. While polysomnography (PSG) and the MSLT are recommended as part of the diagnostic evaluation of narcolepsy in children and adolescents, caution must be used, as there are no standards regarding interpretation of MSLTs in prepubertal children, and normative values in children less than 8 years old do not currently exist. In addition, there are reports of prepubertal children experiencing sleep attacks and cataplexy months prior to the appearance of sleep onset rapid eye movement (SOREMs) on the MSLT, suggesting it may be a less sensitive test than in adults. Children should have 8 or more hours of sleep (as contrasted with at least 6 in adults) confirmed by PSG on the night before MSLT testing. Pediatric narcolepsy often results in severe learning and social impairment; narcolepsy awareness efforts should be aimed at parents, pediatric health care providers, school professionals, and children/adolescents themselves. In this symposium, experts will address best practices in the diagnosis and treatment of narcolepsy, and explore hypersomnia mimics of narcolepsy in children.

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