Autonomic Regulation in Prader-Willi Syndrome

Study Overview
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Brief Summary
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To evaluate autonomic regulation in patients with Prader-Willi syndrome with sleep-disordered breathing.

Detailed Description
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To evaluate the relationship between narcolepsy and sleep-disordered breathing, the natural course of untreated or treated sleep-disordered breathing and its impacts on autonomic regulation in patients with Prader-Willi syndrome with sleep-disordered breathing.

Official Title
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Impact of Sleep-disordered Breathing on Autonomic Regulation in Patients With Prader-Willi Syndrome

Conditions
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Autonomic Dysfunction

Intervention / Treatment
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* Diagnostic Test: Polysomnography
* Diagnostic Test: Multiple sleep latency test
* Other: Continuous positive airway pressure


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: 7-75 y/o Prader-Willi syndrome patients or healthy subjects can perform polysomnography, multiple sleep latency test orthostatic methods for autonomic function test can perform pulmonary function test. Exclusion Criteria: less than y/o, or larger then 75 y/o cannot perform polysomnography, multiple sleep latency test cannot orthostatic methods for autonomic function test cannot perform pulmonary function test use of sedatives or drugs affecting autonomic function

Ages Eligible for Study
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Minimum Age: 7 Years
Maximum Age: 75 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Control<br>Healthy subjects, 7-75 y/o | Diagnostic Test: Polysomnography<br>* overnight polysomnography recording at least six hours<br>* Other names: PSG;Diagnostic Test: Multiple sleep latency test<br>* daytime naps at least four to five naps to evaluate daytime sleepiness and narcolepsy<br>* Other names: MSLT;|
| Study<br>7-75 y/o, diagnosed with Prader-Willi syndrome. | Diagnostic Test: Polysomnography<br>* overnight polysomnography recording at least six hours<br>* Other names: PSG;Diagnostic Test: Multiple sleep latency test<br>* daytime naps at least four to five naps to evaluate daytime sleepiness and narcolepsy<br>* Other names: MSLT;Other: Continuous positive airway pressure<br>* continuous positive airway pressure treatment for sleep-disordered breathing<br>* Other names: CPAP;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Narcolepsy prevalence | Compare the narcolepsy prevalence amount healthy and Prader-Willi syndrome with or without sleep-disordered breathing | 1day | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| The natural course and its impact of untreated sleep-disordered breathing on autonomic regulation in patients with Prader-Willi syndrome. | Autonomic function measurements (low frequency power and high frequency power, presented as normalized units, and the overall autonomic regulation presented as Low frequency/high frequency ratio) using the EKG signal extracting from overnight polysomnography recording raw data among Prader-Willi patients with untreated sleep-disordered breathing at baseline and after a period of follow-up. | 12 months |