Developmental trends of sleep‐disordered breathing in Prader‐Willi syndrome: The role of obesity

Abstract

Polysomnographic recordings of 43 children and adults with Prader-Willi syndrome (PWS) were inspected and classified into 5 age groups. The effect of age and body mass index (BMI) on measures of breathing, oxygen saturation, and sleep efficiency were analyzed. Body mass index (BMI) increased significantly between early childhood and preadolescent groups. Subjecting the data to analysis of variance showed an overall significant effect of BMI but no age effect on breathing parameters and oxygen saturation. Increased BMI was associated with decreased oxygen saturation and with higher apnea/hypopnea index. Sleep efficiency index was significantly lower in adults than in young children, preadolescent, and adolescent groups. These findings emphasize the role of obesity in the development of sleep-related breathing abnormalities and nocturnal oxygen desaturation in patients with PWS.

Sleep-Disordered Breathing in Prader-Willi Syndrome and its Association with Neurobehavioral Abnormalities

“…[5][6][7] The sleep-disordered breathing was mainly obstructive in type, as evidenced by a high frequency of snoring, inspiratory flow limitation, and obstructive apneas and hypopneas occurring mainly in REM sleep. In keeping with previous reports, 1,6,9 there was a correlation between obesity and features of sleep apnea, such as sleep hypoxemia and sleep disruption. Sustained partial upper airway obstruction was present, as evidenced by persistent snoring and inspiratory flow limitation in many subjects, even those without significant sleep apnea.…”

“…(J Pediatr 2005;147:823-9) A lthough early reports suggested that sleep-disordered breathing was uncommon in Prader-Willi syndrome (PWS), 1-4 most recent studies [5][6][7] (although not all 8 ) have found that it is in fact common, especially during rapid eye movement (REM) sleep. 5,6,9 However, uncertainty remains regarding the nature and causes of sleepdisordered breathing in PWS.…”

“…(J Pediatr 2005;147:823-9) A lthough early reports suggested that sleep-disordered breathing was uncommon in Prader-Willi syndrome (PWS), 1-4 most recent studies [5][6][7] (although not all 8 ) have found that it is in fact common, especially during rapid eye movement (REM) sleep. 5,6,9 However, uncertainty remains regarding the nature and causes of sleepdisordered breathing in PWS. 10 Reduced ventilatory 7,[11][12][13][14] and arousal responses 12,15 to hypoxia and hypercapnia in PWS could potentiate sleep hypoventilation, contribute to REM central apneas, 8 or cause a prolongation of obstructive events.…”

“…Obesity, particularly morbid obesity, is common in PWS and has been associated with sleep desaturation 2 and sleep apnea-hypopnea. 1,6 Daytime hypersomnolence is a hallmark of PWS and has been associated with sleeponset REM sleep in daytime naps. 1,16 Hypersomnolence occurs in the absence of sleepdisordered breathing in PWS, 1,9 and successful treatment of sleep-disordered breathing improves, 5 but does not eliminate, hypersomnolence.…”

Absence ofNdn, Encoding the Prader-Willi Syndrome-Deleted Genenecdin, Results in Congenital Deficiency of Central Respiratory Drive in Neonatal Mice

“…Inset shows whole-cell and integrated nerve recordings during a single inspiratory burst. sleepiness in childhood and adulthood that is characteristic of PWS (Hertz et al, 1995). Aside from one report showing reduced number of oxytocin neurons in the hypothalamic paraventricular nucleus, no abnormal pathological findings have been noted in PWS individuals at autopsy (Swaab et al, 1995).…”