Prader-Willi Syndrome and Sleep-Disordered Breathing

Abstract

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.