Prader-Willi Syndrome and Sleep-Disordered Breathing

CME Educational Objectives 1. Identify commonly observed sleep abnormalities in patients with Prader-Willi Syndrome (PWS). 2. Discuss the effects of growth-hormone therapy on sleep-disordered breathing in patients with PWS. 3. Identify other sleep-related abnormalities encountered in patients with PWS. Prader-Willi Syndrome is a complex neurogenetic disorder characterized by appetite dysregulation, obesity with decreased muscle mass and increased fat mass, behavioral problems, various endocrinopathies, and sleep and respiratory abnormalities. Respiratory issues include both central and obstructive sleep apnea, excessive daytime sleepiness, narcolepsy, and impaired ventilatory control. There is some controversy as to whether growth hormone treatment, which is the standard of care for patients with Prader-Willi Syndrome, exacerbates or ameliorates the sleep-disordered breathing issues associated with this syndrome. The natural history of sleep and breathing issues and the issues surrounding growth hormone treatment on sleep-disordered breathing are discussed.