van Hilten J J, Kerkhof G A, van Dijk J G, Dunnewold R, Wintzen A R
Department of Neurology and Clinical Neurophysiology, Academic Hospital, Leiden, The Netherlands.
J Neurol Sci. 1993 Jan;114(1):68-75. doi: 10.1016/0022-510x(93)90051-y.
Ten patients with myotonic dystrophy (MyD) and excessive daytime sleepiness (EDS) were studied. Daytime sleepiness was assessed by means of a subjective alertness rating scale, multiple sleep latency tests and auditory event-related potentials. In addition, the diurnal pattern of daytime sleepiness and ultradian rhythm characteristics of nocturnal sleep were assessed. The multiple sleep latency tests and the P300 component of the auditory event-related potentials gave evidence of EDS, whereas the subjective alertness ratings did not. However, the alertness ratings showed a significantly reduced circadian periodicity as compared with controls. Consistent with this, no time-of-day effect was observed for the multiple sleep latency test outcomes. The ultradian rhythm characteristics of nocturnal sleep indicated a prolonged mean cycle duration and decreased stability of NREM/REM cycle. Moreover, the temporal structure of REM sleep showed a pattern similar to that of subjects who develop free-running rhythms when living in temporal isolation. These findings suggest that EDS in MyD reflects a complex and wide-spread malfunction of the circadian and ultradian timing system.
对10名患有强直性肌营养不良（MyD）且白天过度嗜睡（EDS）的患者进行了研究。通过主观警觉性评分量表、多次睡眠潜伏期测试和听觉事件相关电位来评估白天嗜睡情况。此外，还评估了白天嗜睡的昼夜模式以及夜间睡眠的超日节律特征。多次睡眠潜伏期测试和听觉事件相关电位的P300成分显示存在EDS，而主观警觉性评分则未显示。然而，与对照组相比，警觉性评分显示昼夜周期性显著降低。与此一致的是，多次睡眠潜伏期测试结果未观察到时间效应。夜间睡眠的超日节律特征表明平均周期持续时间延长，非快速眼动/快速眼动周期稳定性降低。此外，快速眼动睡眠的时间结构显示出一种模式，类似于那些在时间隔离中生活时出现自由运行节律的受试者。这些发现表明，MyD中的EDS反映了昼夜节律和超日计时系统的复杂且广泛的功能障碍。
