Objectives: To determine the prevalence and type of sleep-disordered breathing among patients with Prader-Willi syndrome (PWS) and its relationship to such neurobehavioral abnormalities as mental retardation, obsessiv...Objectives: To determine the prevalence and type of sleep-disordered breathing among patients with Prader-Willi syndrome (PWS) and its relationship to such neurobehavioral abnormalities as mental retardation, obsessive-compu-lsive behavior, and conduct disorders. Study design: Polysomnography (PSG)-studies were conducted in 13 unselected subjects with PWS (age 1.5 to 28 years). PSG results were compared with tests of behavior and cognition (Development Behavior Checklist DBC , Auditory Continuous Performance Test ACPT , and Wechsler Intelligence Scale appropriate for age). Results: Nine of 13 (69%) subjects had > 10 apneas and hypopneas per hour of sleep. Apart from a 2-year-old subject with normal body weight who demonstrated severe central hypopnea in rapid eye movement sleep, the sleep-breathing disturbance was due to upper airway obstruction. Age-adjusted body mass index was associated with more severe hypoxemia during sleep (min SaO2, r=-.87, P < .005) and more sleep disruption (arousalshour of sleep, r= .62, P < .05; sleep efficiency, r=-.66, P < .05). Increasing severity of obstructive sleep apnea (OSA) or sleep disturbance was associated with daytime inactivitysleepiness and autistic-relating behavior (DBC) and with impulsiveness (ACPT). Unexpectedly, sleep hypoxemia appeared to be predictive of increased performance IQ. Conclusions: OSA is prevalent among subjects with PWS and is associated with increased body mass, daytime inactivity sleepiness, and some behavioral disturbances.展开更多
文摘Objectives: To determine the prevalence and type of sleep-disordered breathing among patients with Prader-Willi syndrome (PWS) and its relationship to such neurobehavioral abnormalities as mental retardation, obsessive-compu-lsive behavior, and conduct disorders. Study design: Polysomnography (PSG)-studies were conducted in 13 unselected subjects with PWS (age 1.5 to 28 years). PSG results were compared with tests of behavior and cognition (Development Behavior Checklist DBC , Auditory Continuous Performance Test ACPT , and Wechsler Intelligence Scale appropriate for age). Results: Nine of 13 (69%) subjects had > 10 apneas and hypopneas per hour of sleep. Apart from a 2-year-old subject with normal body weight who demonstrated severe central hypopnea in rapid eye movement sleep, the sleep-breathing disturbance was due to upper airway obstruction. Age-adjusted body mass index was associated with more severe hypoxemia during sleep (min SaO2, r=-.87, P < .005) and more sleep disruption (arousalshour of sleep, r= .62, P < .05; sleep efficiency, r=-.66, P < .05). Increasing severity of obstructive sleep apnea (OSA) or sleep disturbance was associated with daytime inactivitysleepiness and autistic-relating behavior (DBC) and with impulsiveness (ACPT). Unexpectedly, sleep hypoxemia appeared to be predictive of increased performance IQ. Conclusions: OSA is prevalent among subjects with PWS and is associated with increased body mass, daytime inactivity sleepiness, and some behavioral disturbances.
