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Sleep Consequences of Prader-Willi Syndrome
Reem Itani1, Emily S Gillett1,2, Iris A Perez3,4
1Department of Pediatric Pulmonology and Sleep Medicine, Children's Hospital Los Angeles, Los Angeles, CA, USA.
Current Neurology and Neuroscience Reports
|February 15, 2023
Summary
Sleep disorders, including breathing disturbances and excessive daytime sleepiness (EDS), are common in Prader-Willi syndrome (PWS). Early diagnosis and treatment of these conditions can improve patient outcomes and survival.
Area of Science:
- Neurology
- Sleep Medicine
- Genetics
Background:
- Prader-Willi syndrome (PWS) is a complex genetic disorder associated with hypothalamic dysfunction.
- Sleep disturbances are a significant and often underdiagnosed comorbidity in PWS patients.
Purpose of the Study:
- To review the impact of sleep disorders on patients with Prader-Willi syndrome.
- To focus on sleep-related breathing disturbances and excessive daytime sleepiness (EDS) in PWS.
Main Methods:
- Literature review of studies on sleep in Prader-Willi syndrome.
- Analysis of recent findings on sleep-related breathing disorders and EDS.
Main Results:
- Central sleep apnea (CSA) can persist in PWS, and nocturnal hypoventilation is common.
- Obstructive sleep apnea (OSA) requires monitoring, especially during growth hormone (GH) therapy.
- Excessive daytime sleepiness (EDS) and narcolepsy are prevalent, with emerging pharmacologic treatments.
Conclusions:
- Sleep disorders are highly prevalent in PWS and require proactive management.
- Effective treatment of sleep-related breathing disorders and EDS is crucial for improving clinical outcomes and survival in PWS.
- Continued surveillance for sleep disturbances is recommended, even with GH therapy.
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