Greaves N, Prince E, Evans D W, Charman T
Islington Primary Care NHS Trust, London, UK.
J Intellect Disabil Res. 2006 Feb;50(Pt 2):92-100. doi: 10.1111/j.1365-2788.2005.00726.x.
Recent research has shown that the range of repetitive behaviour seen in individuals with Prader-Willi syndrome (PWS) extends beyond food-related behaviour.
The presence and intensity of repetitive, rigid and routinized behaviour in children with PWS was compared with that seen in children with another neurodevelopmental condition in which repetitive behaviour is common: children with autism. Parents completed the Childhood Routines Inventory (CRI).
Contrary to our predictions, controlling for developmental level, children with PWS and children with autism showed similar levels of repetitive and ritualistic behaviour overall and on the two CRI factors measuring 'just right' and 'repetitive' behaviour. Indeed, the majority of the sample of parents of children with PWS endorsed most items on the CRI. However there was some specificity at the level of individual items with parents of children with PWS more frequently endorsing an item on 'collecting and storing objects' and parents of children with autism more frequently endorsing 'lining up objects', 'has a strong preference for certain foods' and 'seems aware of detail at home'.
These findings confirm the range of repetitive behaviours that form part of the behavioural phenotype of PWS, including insistence on sameness and 'just right' behaviours, and uncover a surprising overlap with those seen in children with autism. Clinical management for children with PWS should include advice and education regarding management of repetitive and rigid behaviour. Future research should investigate whether the repetitive behaviours that form part of the behavioural phenotype of both PWS and autism are associated with a common neuropsychological, neurotransmitter or genetic origin.
最近的研究表明,普拉德-威利综合征(PWS)患者的重复行为范围超出了与食物相关的行为。
将PWS患儿重复、刻板和程式化行为的存在情况及强度,与另一种常见重复行为的神经发育障碍患儿(自闭症患儿)进行比较。家长完成儿童日常活动量表(CRI)。
与我们的预测相反,在控制发育水平后,PWS患儿和自闭症患儿在总体上以及在测量“恰到好处”和“重复”行为的两个CRI因子上,表现出相似水平的重复和仪式化行为。事实上,PWS患儿家长样本中的大多数人认可CRI上的大多数项目。然而,在个别项目层面存在一些特异性,PWS患儿家长更频繁地认可“收集和储存物品”项目,而自闭症患儿家长更频繁地认可“排列物品”、“对某些食物有强烈偏好”和“似乎注意到家中细节”。
这些发现证实了构成PWS行为表型一部分的重复行为范围,包括坚持一致性和“恰到好处”行为,并发现与自闭症患儿的行为存在惊人的重叠。对PWS患儿的临床管理应包括关于重复和刻板行为管理的建议和教育。未来的研究应调查构成PWS和自闭症行为表型一部分的重复行为是否与共同的神经心理学、神经递质或遗传起源有关。