Polysomnographic characteristics in patients with Prader–Willi syndrome

Abstract

To evaluate the occurrence of sleep-disordered breathing and to clarify the characteristics of sleep among patients with Prader-Willi syndrome (PWS). Overnight continuous EEG-polysomnographic studies were performed in 30 patients with PWS (16 males and 14 females; mean age, 7.4 +/- 4.1 years; age range, 1-19 years) unselected for sleep disturbance. The baseline arterial oxygen saturation (SpO2) was 96.6 +/- 0.6%, with a nadir of 77.2 +/- 10.2%. The rapid eye movement (REM) latency was 67.4 +/- 30.0 min. The percent of total sleep time spent in sleep stages 1, 2, slow wave, and REM were 13.1 +/- 8.2%, 41.9 +/- 10.5%, 21.5 +/- 9.4%, and 21.1 +/- 5.7%, respectively. The respiratory disturbance index (RDI) was 5.8 +/- 3.7/hr and desaturation index (DI) was 8.1 +/- 7.3/hr, respectively. Age-adjusted BMI was associated with more severe hypoxemia during sleep (baseline SpO2, r = -0.53, P < 0.01; nadir SaO2, r = -0.65, P < 0.01; RDI, r = 0.37, P < 0.05; DI, r = 0.53, P < 0.01) and more sleep disruption (arousal index, r = 0.46, P < 0.01). There were no significant associations between gender or genotype pattern (deletion vs. uniparental disomy) and the results of polysomnography. Sleep hypoxemia and sleep disruption are more prevalent in patients with PWS than in normal children. Obesity in these patients is associated with more severe sleep-disordered breathing.

Clinically Important Age-Related Differences in Sleep Related Disordered Breathing in Infants and Children with Prader-Willi Syndrome

“…Data on prevalence of sleep apnea in the PWS population vary greatly [24], prevalence is reported to range from zero in older children and adults [32] to 100% in infant subjects with PWS [30]. This variability is at least partly explained by differences in the specific characteristics of the sleep apnea [28]. Variable and wide age ranges, varying diagnostic criteria and a potential referral bias may contribute to differences in SDB prevalence estimations between studies.…”

Sleep disordered breathing in patients with Prader-Willi syndrome: A multicenter study

“…2 The prevalence of SDB, OSA, and central apnea is significantly higher in patients with PWS as compared to healthy controls. [3][4][5][6][7][8] OSA is the most common presentation of SDB in PWS with a prevalence ranging from 0 to 100%, depending on patient selection and OSA criteria. [3][4][5][6][7][8][9][10][11][12] The actual prevalence of SDB is difficult to define due to the small number of patients reported, the variable inclusion criteria, the wide age ranges, and the lack of multicenter studies.…”

Rebuttal: Turf Wars and Silos—The effect of disruptive technologic innovation in the treatment of cerebral and peripheral vascular disease

“…Overnight sleep studies were conducted at the Mackay Memorial Hospital Sleep Laboratory 11. All recordings were performed by using Embla Systems (Somnologica Studio, Broomfield, CO).…”