Hertz G, Cataletto M, Feinsilver S H, Angulo M
Sleep Disorders Center, Winthrop University Hospital, Mineola, NY 11501.
Sleep. 1993 Jun;16(4):366-71. doi: 10.1093/sleep/16.4.366.
Patients with Prader Willi syndrome (PWS) often complain of daytime hypersomnolence. Because of reported daytime sleepiness and high prevalence of morbid obesity, these patients have been considered at risk for sleep related disordered breathing, but polysomnographic studies have been limited. We evaluated sleep and breathing polysomnographically in 24 PWS patients including 15 adults and 9 children. All adult patients completed MSLT testing on the day following the nocturnal sleep study. Both adult and children groups showed little or no sleep apnea, but REM related oxygen desaturation was quite common, its severity significantly correlated with increased obesity. Sleep patterns in both groups showed abnormal REM sleep cycles with variable REM latency (at times significantly shortened) and fragmented REM sleep with multiple brief REM periods. REM sleep abnormalities were still present in some patients without REM related desaturation. As a group, patients with PWS demonstrated pathological somnolence as measured by MSLT, which correlated with nocturnal sleep efficiency but not with nocturnal REM latency. It is hypothesized that the abnormal sleep findings in PWS reflect an underlying hypothalamic dysfunction characteristic of this syndrome.
普拉德-威利综合征（PWS）患者常主诉日间嗜睡。鉴于有报道称这些患者存在日间困倦且病态肥胖的患病率较高，他们被认为有睡眠相关呼吸障碍的风险，但多导睡眠图研究一直有限。我们对24例PWS患者进行了多导睡眠图睡眠和呼吸评估，其中包括15名成人和9名儿童。所有成年患者在夜间睡眠研究后的次日完成了多次睡眠潜伏期试验（MSLT）。成人组和儿童组均显示很少或没有睡眠呼吸暂停，但快速眼动（REM）相关的氧饱和度下降相当常见，其严重程度与肥胖增加显著相关。两组的睡眠模式均显示REM睡眠周期异常，REM潜伏期可变（有时显著缩短），且REM睡眠碎片化，有多个短暂的REM期。一些没有REM相关氧饱和度下降的患者仍存在REM睡眠异常。总体而言，PWS患者经MSLT测量显示存在病理性嗜睡，这与夜间睡眠效率相关，但与夜间REM潜伏期无关。据推测，PWS患者异常的睡眠表现反映了该综合征潜在的下丘脑功能障碍。
