Psychopathology and behavior problems in children and adolescents with Williams syndrome: Distinctive relationships with cognition

Abstract

Several studies have documented the high prevalence of psychopathology and behavior problems in Williams syndrome (WS). However, the links between cognitive development and such symptoms need further clarification. Our study aims to expand current knowledge on levels of behavior problems and its links to cognition in a sample of Brazilian individuals with WS. A total of 25 children and adolescents with WS and their parents participated in this study. The participants' IQs were assessed with the Wechsler Scales of Intelligence (for children or adults) and parental reports of psychopathology/behavior problems were collected using the Child Behavior Checklist (CBCL). The presence of clinically significant attention problems was a main feature in our sample of children and adolescents with WS. In the children, higher IQ scores were found to be significantly associated with less externalizing problems, while in the adolescents cognitive abilities were found to be associated with less internalizing symptoms. These results provide further insight into the links between psychopathology and behavior problems and cognitive abilities in WS, and suggest the need to take age into consideration when analyzing such relationships.

Association between speech–language, general cognitive functioning and behaviour problems in individuals with Williams syndrome

“…Regarding behavioural problems, a higher percentage of individuals were in the borderline/clinical range on the CBCL Total Problems scale (Teixeira et al ; Klein‐Tasman et al ; Osório et al ), and as predicted, age effects were rare (Pérez‐García et al ). We found a negative association only between CA and the Aggressive Behaviour scale.…”

“…Pérez‐García et al () replicated a negative correlation between IQ and Thought Problems for the American sample, but not for the Spanish sample. According to Osório et al (), higher verbal IQ scores have a protective effect on internalising symptoms in adolescents and on externalising symptoms in children with WS.…”

Cognitive and behavioral profile of Williams Syndrome toddlers

“…The test covers ages between 2.5 to 90 years and consists of 125 items, plus 5 training items, with four pictures on each screen. In the test, the child should point to the figure that corresponds to the word he/she hears; c. Child Behavior Checklist for Children Ages1 ½ to 5 (CBCL/1.5-5), and Child Behavior Checklist for Children Ages6-18 (CBCL/6-18) (16) : The checklists are based on a multi-axial approach. The behavioral problem items from both questionnaires are distributed in five scales: syndromes scales, DSM oriented scale, internalizing, externalizing and total problems scales).…”

“…Brazilian studies have focused mainly on other ages, such as children and adolescents (5,13) . The early identification of phenotypic indicators that can later interfere with the literacy, adaptation and socialization of the infants is crucial.…”

“…Standardized intelligence assessments report overall deficits in cognitive abilities indicative of mild to severe intellectual disability (3,4) . The distinctive behavioral and clinical characteristics of toddlers and infants with WS compared to typical children and other individuals with neurodevelopmental disorders associated with intellectual disability (ID) are emotional and behavior problems (EBP), fears, different psychiatric conditions as specific phobias, generalized anxiety disorder, and attention-deficit/hyperactivity disorder (ADHD) (5) , appetitive drive toward social engagement and heightened approachability, excessive social responsiveness, and lack of selectivity in interpersonal contacts, difficulties in emotion processing with decreased recognition of negative social signals, emotional responsivity, including enhanced empathic display and reaction (6) . Previous studies have also demonstrated severe hyperacusis during early childhood that may decrease with age (7) .…”