Harris J C, Allen R P
Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, Md, USA.
Arch Pediatr Adolesc Med. 1996 Dec;150(12):1288-93. doi: 10.1001/archpedi.1996.02170370066011.
To assess nighttime and daytime sleep patterns in patients with Prader-Willi syndrome and to examine the effects of weight change on excessive daytime sleepiness in patients with this disorder.
Case series (within-subject design).
A university sleep disorders center.
Eight patients (5 males and 3 females), ranging in age from 5.5 to 21 years, who met the diagnostic criteria for Prader-Willi syndrome.
Overnight sleep polysomnographic recording and daytime Multiple Sleep Latency Test. Four of the 8 patients were restudied after their weight had changed.
Changes in the sleep disordered breathing rate and Multiple Sleep Latency Test measures.
Sleep-disordered breathing occurred in all patients and was principally characterized by obstructive hypoventilation or episodes of apnea that occurred primarily during rapid eye movement sleep. After weight reduction, 3 patients had respiratory values that were within the broad normal range (disordered breathing rate, < 15 breaths per hour). Statistically significant (P < .05) weight loss effects occurred during nonrapid eye movement sleep (decrease with weight loss, F = 6.243). Excessive daytime sleepiness was documented in 6 of 7 patients who completed the Multiple Sleep Latency Test. Excessive daytime sleepiness was not consistently correlated with body weight or any of the nocturnal sleep variables.
A sleep-related breathing disorder occurred during rapid eye movement and nonrapid eye movement sleep and improved with weight change in patients with Prader-Willi syndrome, emphasizing the importance of weight reduction in clinical management. However, excessive daytime sleepiness persisted despite a reduction in sleep-disordered breathing after weight loss, suggesting a primary disturbance of sleep. Our findings provide additional support for the view that primary hypersomnia is a characteristic feature of the Prader-Willi syndrome.
评估普拉德-威利综合征患者的夜间和白天睡眠模式，并研究体重变化对该疾病患者日间过度嗜睡的影响。
病例系列研究（受试者自身对照设计）。
一所大学睡眠障碍中心。
8例患者（5例男性，3例女性），年龄在5.5至21岁之间，符合普拉德-威利综合征的诊断标准。
夜间睡眠多导睡眠图记录和白天多次睡眠潜伏期测试。8例患者中有4例在体重变化后再次接受研究。
睡眠呼吸障碍率和多次睡眠潜伏期测试指标的变化。
所有患者均出现睡眠呼吸障碍，主要表现为阻塞性通气不足或主要在快速眼动睡眠期出现的呼吸暂停发作。体重减轻后，3例患者的呼吸指标在广泛的正常范围内（呼吸障碍率，<15次/小时）。在非快速眼动睡眠期出现了具有统计学意义（P<.05）的体重减轻效应（随体重减轻而降低，F=6.243）。7例完成多次睡眠潜伏期测试的患者中有6例记录到日间过度嗜睡。日间过度嗜睡与体重或任何夜间睡眠变量均无一致的相关性。
普拉德-威利综合征患者在快速眼动和非快速眼动睡眠期均出现与睡眠相关的呼吸障碍，体重变化可使其改善，这强调了体重减轻在临床管理中的重要性。然而，尽管体重减轻后睡眠呼吸障碍有所减轻，但日间过度嗜睡仍然存在，提示存在原发性睡眠障碍。我们的研究结果为原发性发作性睡病是普拉德-威利综合征的特征性表现这一观点提供了更多支持。
