夜间抽搐:神经肌肉疾病或脑瘫儿童睡眠中的周期性肢体运动

L Nisbet, G Nixon, M Davey
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摘要

神经肌肉疾病(NMD)或脑瘫(CP)患儿经常报告睡眠不良,而通气往往是临床关注的焦点。周期性肢体运动(PLMs)在儿科人群中经常被误诊(在临床参考研究中患病率为5-8%),在多达33%的唐氏综合征儿童中发生。我们评估了NMD或CP患儿PLMs的患病率。方法回顾性回顾2005-2022年间NMD(包括杜氏肌营养不良症、肌强直性营养不良症和脊髓性肌萎缩症)或CP患儿的第一张多导睡眠图和腿肌电图。结果238例患儿(NMD 124例,CP 114例)经同意行腿部肌电图检查。72例(30%)为女性,中位年龄9y(范围1 ~ 18y), BMI z-score 0.4 (-3.5 ~ 2.7), RDI 3.5/h (0 ~ 100/h),性唤起指数11.7/h (1.3 ~ 65.6/h)。PLM指数中位数为0(范围0-33/h), PLM唤醒率为0(0-74%)。NMD组和CP组PLM升高(5/h)的发生率分别为9.7%和10.5%,中位PLM觉醒率分别为8.5%和4.5%。PLMs升高或不升高的患者在年龄和性别上没有差异(p < 0.05)。与一般儿科人群相比,NMD和CP患儿PLM指数升高的发生率更高,但低于唐氏综合征患儿。重要的是,睡眠障碍不能被忽视,因为识别和治疗可能有助于改善这一人群的睡眠结果。需要进一步的研究来了解PLMs在这一人群中的病理生理特征。
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O020 Twitch in the Night: Periodic Limb Movements during Sleep in Children with Neuromuscular Disease or Cerebral Palsy
Abstract Introduction Poor sleep is frequently reported in children with neuromuscular diseases (NMD) or cerebral palsy (CP) however ventilation is often the clinical focus. Periodic limb movements (PLMs) are frequently underdiagnosed in the paediatric population (prevalence of 5-8% in clinic-referred studies) and occur in up to 33% of children with Down syndrome. We assessed the prevalence of PLMs in children with NMD or CP. Methods Retrospective review of the first polysomnogram with leg electromyography in children with NMD (including Duchenne muscular dystrophy, myotonic dystrophy, and spinal muscular atrophy) or CP between 2005-2022. Results Leg electromyography was available in 238 children (124 NMD, 114 CP) with consent. 72 (30%) were female with a median age 9y (range 1-18y), BMI z-score 0.4 (-3.5 to 2.7), RDI 3.5/h (0-100/h) and arousal index of 11.7/h (1.3-65.6/h). Median PLM index was 0 (range 0-33/h) with %PLM arousals 0 (0-74%). The prevalence of elevated PLMs (&gt;5/h) was 9.7% and 10.5% in the NMD and CP groups respectively, with median PLM arousals of 8.5% and 4.5% respectively. There were no differences in age or sex between those with or without elevated PLMs (p&gt;0.05). Discussion Elevated PLM index occurred at a higher prevalence in children with NMD and CP than reported in the general paediatric population, though at lower rates than in Down syndrome. It is important that PLMs are not overlooked as identification and treatment may help improve sleep outcomes in this population. Further research is required to understand the pathophysiology of PLMs specifically in this population.
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