Distinctiveness and correlates of maladaptive behaviour in children and adolescents with Smith-Magenis syndrome
Distinctiveness and correlates of maladaptive behaviour in children and adolescents with Smith-Magenis syndrome
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DOI：
10.1046/j.1365-2788.1998.4260481.x
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发表时间：
1998-12-01
影响因子：
3.6
通讯作者：
Smith, ACM
中科院分区：
文献类型：
--
作者：
Dykens, EM;Smith, ACM
这项分为两部分的研究调查了35名患有Smith-Magenis综合征（一种由17号染色体间质缺失引起的发育障碍）的儿童和青少年的适应性不良行为的独特性和相关因素。研究1比较了35名患有史密斯-马格尼斯综合征的儿童与年龄和性别匹配的普瑞德-威利综合征和混合智力残疾儿童的儿童行为检查表得分。患有史密斯-马格尼斯综合症的受试者明显比其他组有更高水平的适应不良行为。虽然有些问题是两组共有的，但12种行为将三组区分开来的准确率为100%。研究2评估了自残和刻板行为的频率和相关性，包括不寻常的特征，如拉指甲、往身体孔里塞东西、自我拥抱和“舔和翻转”行为。与其他类型的自伤行为相比，猛拽指甲和插入身体的行为不太常见，而自我拥抱和“舔和翻转”的刻板印象在大约一半的样本中出现。虽然年龄和延迟程度与问题行为相关，但睡眠障碍是适应不良行为的最强预测因子。本文讨论了Smith-Magenis综合征和Prader-Willi综合征之间的临床诊断歧义以及干预的意义。
This two-part study examines the distinctiveness and correlates of maladaptive behaviour in 35 children and adolescents with Smith-Magenis syndrome, a developmental disorder caused by an interstitial deletion of chromosome 17 (p11.2). Study I compares Child Behavior Checklist scores in 35 children with Smith-Magenis syndrome to age- and gender-matched subjects with Prader-Willi syndrome and mixed intellectual disability. Subjects with Smith-Magenis syndrome had significantly higher levels of maladaptive behaviour than the other groups. Although some problems were shared across groups, 12 behaviours differentiated the three groups with 100% accuracy. Study 2 assessed the frequency and correlates of self-injurious and stereotypical behaviours, including unusual features such as nail-yanking, inserting objects into bodily orifices, self-hugging and a 'lick-and flip' behaviour. Nail-yanking and bodily insertions were less common than other types of self-injury, and self-hugs and the 'lick-and flip' stereotypies were seen in about half the sample. Although age and degree of delay were correlated with problem behaviours, sleep disturbance emerged as the strongest predictor of maladaptive behaviour. The implications are discussed for clinical diagnostic ambiguities between the Smith-Magenis and Prader-Willi syndromes, and for intervention.