BACKGROUND: The prevalence, phenomenology aetiology and correlates of four forms of challenging behaviour in 32 children and adults with Smith-Magenis syndrome (SMS) were investigated. METHODS: Cognitive assessments, questionnaires and semi-structured interviews were used to gather data on intellectual disability, verbal and physical aggression, destructive behaviour and self-injury and on characteristics known to be associated with aggression. RESULTS: Aggression in SMS was more prevalent (87%), but not more severe than aggression in contrast groups. Aggressive behaviour was more frequently associated with environmental contingencies (e.g. attention, escape and access to tangibles) than self-injury and destructive behaviours. Severity of challenging behaviours was associated with high impulsivity. CONCLUSION: Aggression is seen in the majority of people with SMS. Results suggest that behavioural disinhibition and operant social reinforcement are associated with the manifestation of aggression.
BACKGROUND: The prevalence, phenomenology aetiology and correlates of four forms of challenging behaviour in 32 children and adults with Smith-Magenis syndrome (SMS) were investigated. METHODS: Cognitive assessments, questionnaires and semi-structured interviews were used to gather data on intellectual disability, verbal and physical aggression, destructive behaviour and self-injury and on characteristics known to be associated with aggression. RESULTS:Aggression in SMS was more prevalent (87%), but not more severe than aggression in contrast groups. Aggressive behaviour was more frequently associated with environmental contingencies (e.g. attention, escape and access to tangibles) than self-injury and destructive behaviours. Severity of challenging behaviours was associated with high impulsivity. CONCLUSION:Aggression is seen in the majority of people with SMS. Results suggest that behavioural disinhibition and operant social reinforcement are associated with the manifestation of aggression.
Authors: Jessica Klusek; Anna W Hunt; Penny L Mirrett; Deborah D Hatton; Stephen R Hooper; Jane E Roberts; Donald B Bailey Journal: J Autism Dev Disord Date: 2015-06
Authors: Maria Nicla Loviglio; Christine R Beck; Janson J White; Marion Leleu; Tamar Harel; Nicolas Guex; Anne Niknejad; Weimin Bi; Edward S Chen; Isaac Crespo; Jiong Yan; Wu-Lin Charng; Shen Gu; Ping Fang; Zeynep Coban-Akdemir; Chad A Shaw; Shalini N Jhangiani; Donna M Muzny; Richard A Gibbs; Jacques Rougemont; Ioannis Xenarios; James R Lupski; Alexandre Reymond Journal: Genome Med Date: 2016-11-01 Impact factor: 11.117