Narcolepsy in childhood

Abstract

Establishing a diagnosis of narcolepsy can be challenging in children, given the non-specific initial symptoms, limited descriptive ability of the child, and variability in reliability of the parents as historians. In the pre-school-age child, it may be hard to distinguish physiologic napping from pathologic sleepiness. In school age children, the combination of inattentiveness, trouble sleeping at night, sleepiness, and hypnagogic hallucinations can be mistaken for depression, emphasizing the need for a thorough history. Sleep logs, nocturnal polysomnography, the multiple sleep latency test (that sometimes needs to be repeated serially over months when the diagnosis is uncertain), and cerebrospinal fluid hypocretin analysis are key diagnostic studies. Management strategies include regularizing sleep-wake schedules, taking planned daytime naps to dissipate sleepiness, and exercising regularly. Parents should monitor the child closely for symptoms of depression. Counseling for emotional support is also suggested. Pharmacotherapy should target the symptom(s) most bothersome to the child. Drug treatment of daytime sleepiness, cataplexy, sleep fragmentation, and comorbid depression is with agents that are used on an off-label basis and are, at best, only modestly effective. © 2010 Springer-Verlag New York.