Friday, September 21, 2018

Clinical characteristics and burden of illness in pediatric patients with narcolepsy

Giuseppe Plazzi MD, PhD, Heather M. Clawges MD and Judith A. Owens MD, MPH.  Clinical Characteristics and Burden of Illness in Pediatric Patients with Narcolepsy.  Pediatric Neurology, in press.


Narcolepsy is a chronic and lifelong neurologic disorder with onset commonly occurring in childhood or adolescence, and affecting approximately 0.025% to 0.05% of the general population. The primary symptom is excessive daytime sleepiness, which is accompanied by cataplexy in 70% of patients. Other common symptoms include sleep paralysis, hallucinations upon falling asleep or waking, and disrupted nocturnal sleep. Narcolepsy is associated with a considerable burden of illness (BOI), which has been well characterized in adults, and is exacerbated by delays in symptom recognition, diagnosis, and intervention. METHODS: This review describes the specific characteristics and BOI of pediatric narcolepsy, using a wide range of published research data. RESULTS: Pediatric narcolepsy presents distinct challenges in diagnosis and management. Narcolepsy symptoms often initially manifest differently in children and adolescents versus adults, which may pose diagnostic dilemmas. Children often respond to sleepiness with irritability, hyperactivity, and poor attention, which may be misinterpreted as misbehavior or neurocognitive sequelae of other conditions. Pediatric cataplexy symptoms may include subtle and unusual facial expressions or choreic-like movements, which are not observed in adults. Insufficient sleep and circadian rhythm disorders presenting with excessive daytime sleepiness are common in adolescents, potentially confounding narcolepsy diagnosis. Pediatric narcolepsy is also associated with comorbidities including rapid weight gain, precocious puberty, and attention deficit hyperactivity disorder, and increased risk for deficits in social functioning, depression, and anxiety. School performance is also typically impaired, requiring special education services. CONCLUSIONS: Thus, the discrete BOI of pediatric narcolepsy underscores the need for prompt and accurate diagnosis, and appropriate treatment of this disorder.

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