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Causal Models of Clinically Significant Behaviors in Angelman, Cornelia de Lange, Prader-Willi and Smith-Magenis Syndromes
Oliver, Chris ; Adams, Dawn ; Allen, Debbie ; Bull, Leah ; Heald, Mary ; Moss, Jo ; Wilde, Lucy ; Woodcock, Kate
2013
关键词SELF-INJURIOUS-BEHAVIOR BRACHMANN-DELANGE SYNDROME SYNDROME DEL 17P11.2 DU-CHAT-SYNDROMES GENOTYPE-PHENOTYPE CORRELATIONS AUTISM SPECTRUM DISORDERS FRAGILE-X-SYNDROME COMPULSIVE BEHAVIOR ENVIRONMENTAL EVENTS GENETIC SUBTYPES
DOI10.1016/B978-0-12-401662-0.00006-3
英文摘要The operant learning theory account of behaviors of clinical significance in people with intellectual disability (ID) has dominated the field for nearly 50 years. However, in the last two decades, there has been a substantial increase in published research that describes the behavioral phenotypes of genetic disorders and shows that behaviors such as self-injury and aggression are more common in some syndromes than might be expected given group characteristics. These cross-syndrome differences in prevalence warrant explanation, not least because this observation challenges an exclusively operant learning theory account. To explore this possible conflict between theoretical account and empirical observation, we describe the genetic cause and physical, social, cognitive and behavioral phenotypes of four disorders associated with ID (Angleman, Cornelia de Lange, Prader-Willi and Smith-Magenis syndromes) and focus on the behaviors of clinical significance in each syndrome. For each syndrome we then describe a model of the interactions between physical characteristics, cognitive and motivational endophenotypes and environmental factors (including operant reinforcement) to account for the resultant behavioral phenotype. In each syndrome it is possible to identify pathways from gene to physical phenotype to cognitive or motivational endophenotype to behavior to environment and back to behavior. We identify the implications of these models for responsive and early intervention and the challenges for research in this area. We identify a pressing need for meaningful dialog between different disciplines to construct better informed models that can incorporate all relevant and robust empirical evidence.; Education, Special; Psychology, Multidisciplinary; SSCI; 4; ARTICLE; 167-211; 44
语种英语
内容类型期刊论文
源URL[http://ir.pku.edu.cn/handle/20.500.11897/404791]  
专题心理与认知科学学院
推荐引用方式
GB/T 7714
Oliver, Chris,Adams, Dawn,Allen, Debbie,et al. Causal Models of Clinically Significant Behaviors in Angelman, Cornelia de Lange, Prader-Willi and Smith-Magenis Syndromes[J],2013.
APA Oliver, Chris.,Adams, Dawn.,Allen, Debbie.,Bull, Leah.,Heald, Mary.,...&Woodcock, Kate.(2013).Causal Models of Clinically Significant Behaviors in Angelman, Cornelia de Lange, Prader-Willi and Smith-Magenis Syndromes..
MLA Oliver, Chris,et al."Causal Models of Clinically Significant Behaviors in Angelman, Cornelia de Lange, Prader-Willi and Smith-Magenis Syndromes".(2013).
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