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Causal Models of Clinically Significant Behaviors in Angelman, Cornelia de Lange, Prader-Willi and Smith-Magenis Syndromes

Research output: Chapter in Book/Report/Conference proceedingChapter

Published
  • Chris Oliver
  • Dawn Adams
  • Debbie Allen
  • Leah Bull
  • Mary Heald
  • Jo Moss
  • Lucy Wilde
  • Kate Woodcock

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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.

Original languageEnglish
Title of host publicationINTERNATIONAL REVIEW OF RESEARCH IN DEVELOPMENTAL DISABILITIES: CHALLENGING BEHAVIOR, VOL 44
EditorsRP Hastings, J Rojahn
Place of PublicationSAN DIEGO
PublisherElsevier Academic Press
Pages167-211
Number of pages45
DOIs
StatePublished - 2013

Publication series

NameInternational Review of Research in Developmental Disabilities
PublisherELSEVIER ACADEMIC PRESS INC
Volume44
ISSN (Print)2211-6095

Research areas

  • 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

ID: 9147424