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儿童期发病的1型强直性肌营养不良症中的睡眠障碍。

Sleep disorders in childhood-onset myotonic dystrophy type 1.

作者信息

Quera Salva Maria-Antonia, Blumen Marc, Jacquette Aurelia, Durand Marie-Christine, Andre Sonia, De Villiers Martine, Eymard Bruno, Lofaso Frédéric, Heron Delphine

机构信息

Sleep Unit, Department of Physiology, Raymond Poincaré Hospital, 104 boulevard Raymond Poincaré, 92380 Garches, France.

出版信息

Neuromuscul Disord. 2006 Oct;16(9-10):564-70. doi: 10.1016/j.nmd.2006.06.007. Epub 2006 Aug 23.

Abstract

Slowness, fatigue, and learning difficulties are common in young patients with myotonic dystrophy type 1. These features may indicate poor sleep quality. The aim of this study was to search for sleep disorders in this population. This prospective study used questionnaires, genetic testing, night-time polysomnography and multiple sleep latency tests to evaluate objective daytime sleepiness. Twenty-one patients were included. Mean age was 15.0+/-3.0. Age of onset of myotonic disorders was after birth and before 10 years old. Age of diagnosis was 12.0+/-2.9. Fatigue was reported by 76% of patients, while somnolence was present in 52%. Sleep was disturbed by numerous microarousals (mean 16.6+/-7.3/h of sleep) caused by abnormal respiratory events (6/21 patients) and/or periodic limb movements (8/21 patients). In young patients with DM1, complaints of fatigue and/or somnolence should lead to a polysomnography to look for sleep apnea syndrome and/or periodic limb movement, which were present in two-thirds of our population.

摘要

迟缓、疲劳和学习困难在1型强直性肌营养不良的年轻患者中很常见。这些特征可能表明睡眠质量较差。本研究的目的是在该人群中寻找睡眠障碍。这项前瞻性研究使用问卷、基因检测、夜间多导睡眠图和多次睡眠潜伏期测试来评估客观的日间嗜睡情况。纳入了21名患者。平均年龄为15.0±3.0岁。强直性疾病的发病年龄在出生后至10岁之前。诊断年龄为12.0±2.9岁。76%的患者报告有疲劳,52%的患者有嗜睡。睡眠受到大量微觉醒的干扰(平均每小时睡眠中出现16.6±7.3次),这些微觉醒由异常呼吸事件(6/21例患者)和/或周期性肢体运动(8/21例患者)引起。在患有DM1的年轻患者中,疲劳和/或嗜睡的主诉应促使进行多导睡眠图检查,以寻找睡眠呼吸暂停综合征和/或周期性肢体运动,在我们的研究人群中,三分之二的患者存在这些情况。

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