杜氏肌肉萎缩症患者与年龄相关的睡眠纺锤体特征

Katharine C Simon, Chelsea Cadle, Neal Nakra, Marni C Nagel, Paola Malerba
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引用次数: 0

摘要

非快速眼动睡眠的大脑振荡,包括慢振荡(SO,0.5-1.5 Hz)和棘波(10-16 Hz),反映了大脑在整个发育过程中的潜在成熟,并与认知有关。因此,与年龄相关的这些节律的出现和电生理特性的变化可以帮助人们深入了解大脑皮层的发育情况,特别是在比较儿科人群和发育正常的同龄人时。我们以前曾评估过杜氏肌营养不良症(DMD)男性患者中与年龄相关的 SOs 变化,发现在 4 至 18 岁期间,SOs 会出现明显的年龄相关性下降。虽然 DMD 主要是一种肌肉疾病,但男性 DMD 患者也可能出现睡眠、认知和皮质异常,这被认为是大脑中的肌营养不良蛋白表达发生改变所致。在这项后续研究中,我们描述了睡眠纺锤体与年龄相关的变化。我们发现,DMD 患者随年龄变化的纺锤体特征,包括密度、频率、振幅和持续时间,与文献报道的发育正常对照组随年龄变化的趋势一致。结合我们之前发现的与年龄相关的SOs下降,我们的结果表明,SOs而非纺锤体是改善DMD患者睡眠的候选干预目标。
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Age-associated sleep spindle characteristics in Duchenne muscular dystrophy.

Brain oscillations of non-rapid eye movement sleep, including slow oscillations (SO, 0.5-1.5 Hz) and spindles (10-16 Hz), mirror underlying brain maturation across development and are associated with cognition. Hence, age-associated emergence and changes in the electrophysiological properties of these rhythms can lend insight into cortical development, specifically in comparisons between pediatric populations and typically developing peers. We previously evaluated age-associated changes in SOs in male patients with Duchenne muscular dystrophy (DMD), finding a significant age-related decline between 4 and 18 years. While primarily a muscle disorder, male patients with DMD can also have sleep, cognitive, and cortical abnormalities, thought to be driven by altered dystrophin expression in the brain. In this follow-up study, we characterized the age-associated changes in sleep spindles. We found that age-dependent spindle characteristics in patients with DMD, including density, frequency, amplitude, and duration, were consistent with age-associated trends reported in the literature for typically developing controls. Combined with our prior finding of age-associated decline in SOs, our results suggest that SOs, but not spindles, are a candidate intervention target to enhance sleep in patients with DMD.

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