Niakan E, Belluomini J, Lemmi H, Summitt R L, Ch'ien L
Ann Neurol. 1979 Sep;6(3):253-7. doi: 10.1002/ana.410060312.
The electrophysiological features of 3 brothers with the classic form of Pelizaeus-Merzbacher disease (PMD) were studied. Two consecutive overnight polygraphic sleep recordings indicated a gross distortion of rapid-eye-movement (REM) sleep for all patients. A lower than normal percentage of REM sleep in these patients was consistent with their retarded intellectual development, which supports current thinking that REM sleep may be a sensitive index of brain function integrity. Non-rapid-eye-movement (NREM) sleep, in contrast to reported findings in 1 patient with PMD, was uniformly characterized by distinct stages in which the electroencephalograms contained frequent vertex waves and spindles. Tests of peripheral nerve conduction velocity, acoustic brainstem reflexes, and visual and auditory evoked potentials did not indicate any abnormalities, nor did electroencephalograms obtained during wakefulness. One patient did have epileptiform spikes and spike waves recorded during an all-night EEG, an unusual finding in a child with cerebral white matter disease.
对3名患有典型型佩利措伊斯-梅茨巴赫病（PMD）的兄弟的电生理特征进行了研究。连续两个夜间的多导睡眠记录显示，所有患者的快速眼动（REM）睡眠严重扭曲。这些患者中REM睡眠的百分比低于正常水平，这与他们智力发育迟缓相一致，这支持了目前的观点，即REM睡眠可能是脑功能完整性的一个敏感指标。与1例PMD患者的报告结果相反，非快速眼动（NREM）睡眠的特征是脑电图中频繁出现顶叶波和纺锤波，各阶段特征明显。外周神经传导速度、听性脑干反射以及视觉和听觉诱发电位测试均未显示任何异常，清醒时的脑电图也未显示异常。1例患者在整夜脑电图记录中出现癫痫样棘波和棘慢波，这在患有脑白质病的儿童中是一个不寻常的发现。
