Obstructive sleep apnea syndrome in Prader-Willi Syndrome: an unrecognized and untreated cause of cognitive and behavioral deficits?
Prader-Willi Syndrome (PWS) is a rare genetic disorder characterized by a range of physical, psychological, and physiological abnormalities. It is also distinguished by the high prevalence of obstructive sleep apnea syndrome (OSAS), i.e., repetitive upper airway collapse during sleep resulting in hypoxia and sleep fragmentation. In non-PWS populations, OSAS is associated with a range of neurocognitive and psychosocial deficits. Importantly, these deficits are at least partly reversible following treatment. Given the findings in non-PWS populations, it is possible that OSAS may contribute to neurocognitive and psychosocial deficits in PWS. The present review examines this possibility. While acknowledging a primary contribution from the primary genetic abnormality to central neural dysfunction in PWS, we conclude that OSAS may be an important secondary contributing factor to reduced neurocognitive and psychosocial performance. Treatment of OSAS may have potential benefits in improving neurocognitive performance and behavior in PWS, but this awaits confirmatory investigation.
Camfferman
Danny
D
Adelaide Institute for Sleep Health, Repatriation General Hospital, Daw Park, Adelaide, South Australia, Australia.
Lushington
Kurt
K
O'Donoghue
Fergal
F
Doug McEvoy
R
R
D000818:Animals; D001526:Behavioral Symptoms / Q000209:etiology*; D003072:Cognition Disorders / Q000209:etiology*; D006801:Humans; D011218:Prader-Willi Syndrome / Q000150:complications*; D020181:Sleep Apnea, Obstructive / Q000209:etiology*