Departamento de Lingüística General, Facultad de Filosofía y Letras, Universidad Autónoma de Madrid, 28049 Madrid, Spain.
Departamento de Filología Española, Facultad de Filosofía y Letras, Universidad Autónoma de Madrid, 28049 Madrid, Spain; Facultad de Ciencias de la Salud, Universidad de Castilla La Mancha, 45600 Talavera de la Reina, Spain; Centro Universitario de Educación Superior (CUNIMAD), 28040 Madrid, Spain.
Res Dev Disabil. 2022 Sep;128:104286. doi: 10.1016/j.ridd.2022.104286. Epub 2022 Jun 29.
The Smith-Magenis syndrome (SMS) shows a collection of neurodevelopmental problems including mild to moderate intellectual disability, change-related anxiety, impulsivity, speech delay, Attention-Deficit/Hyperactivity Disorder (ADH) and sleep disturbances. Sleep disorders, when present, have been treated in several populations with consecutive improvements in cognitive and behavioral aspects.
To better understand the existing relationships between sleep disturbances and behavioral problems in SMS syndrome this study describes the sleep and behavior problems in the SMS and explores the possible relation between both.
17 individuals with SMS (50% males; 11.2 ± 4.9 years old) and 12 individuals with typical development (50% male; 11.1 ± 4.4 years old) were investigated using the Sleep Disturbance Scale for Children and the Child Behavior Checklist.
A high percentage (60%) of individuals with SMS have an indication of sleep disorders, being the most frequent disorders the sleep-wake transition disorders, and disorders of initiating and maintaining sleep with sleep latency higher than acceptable and total sleep time below acceptable. More than 94% of the SMS group presented clinical or borderline scores on the total behavioral problems scale. The most common behavioral problems were Externalizing Problems, Thought and Attention, ADH and Aggressive problems. There was a positive correlation between disorders of initiating and maintaining sleep, sleep-wake transition disorders, disorders of arousal, disorders of excessive somnolence and behavioral problems.
The worse the sleep disturbances investigated, the more severe the behavioral problems characteristics reinforcing the importance to address the sleep problems in the treatment of SMS individuals.
史密斯-马根尼斯综合征 (SMS) 表现为一系列神经发育问题，包括轻度至中度智力障碍、与变化相关的焦虑、冲动、言语延迟、注意力缺陷多动障碍 (ADHD) 和睡眠障碍。在多个群体中，睡眠障碍通过连续改善认知和行为方面得到了治疗。
为了更好地了解 SMS 综合征中睡眠障碍与行为问题之间的现有关系，本研究描述了 SMS 中的睡眠和行为问题，并探讨了两者之间可能的关系。
对 17 名 SMS 患者（50%为男性；11.2±4.9 岁）和 12 名具有典型发育的个体（50%为男性；11.1±4.4 岁）进行了调查，使用儿童睡眠障碍量表和儿童行为检查表。
SMS 患者中有高比例（60%）存在睡眠障碍，最常见的障碍是睡眠-觉醒转换障碍以及入睡和维持睡眠障碍，睡眠潜伏期高于可接受水平，总睡眠时间低于可接受水平。SMS 组超过 94%的个体在总行为问题量表上出现临床或边缘分数。最常见的行为问题是外化问题、思维和注意力、ADHD 和攻击问题。入睡和维持睡眠障碍、睡眠-觉醒转换障碍、觉醒障碍、过度嗜睡障碍与行为问题之间存在正相关。
调查中睡眠障碍越严重，行为问题特征越严重，这进一步强调了在治疗 SMS 个体时解决睡眠问题的重要性。
