Problem behaviour and psychosocial functioning in young children with Williams syndrome: parent and teacher perspectives.

BACKGROUND: There is sparse literature about problem behaviour in young children with Williams syndrome (WS) and little consideration of the perspectives of multiple respondents. METHODS: Problem behaviour of 35 children with WS ages 2 to 6 was examined based on both parent and teacher report using the Achenbach preschool forms. RESULTS: The most prominent areas of difficulty based on both parent and teacher report were attention problems, pervasive developmental problems and emotion reactivity difficulties. Some rater differences were observed; most notably, teachers reported more externalising behaviour problems including more aggressive behaviour, Attention Deficit/Hyperactivity problems and Oppositional Defiant problems than did parents. Few relations to intellectual functioning, age or gender were observed. DISCUSSION: Some aspects of problem behaviour evident in older children (e.g. attention problems, social problems) are also apparent for young children with WS, while other areas are less prominent (e.g. anxiety). The implications of the findings for understanding the behavioural phenotype associated with WS are discussed.

Predictors of specific phobia in children with Williams syndrome.

BACKGROUND: Specific phobia (SP) is the most common anxiety disorder among children with Williams syndrome (WS); prevalence rates derived from Diagnostic and Statistical Manual of Mental Disorders-based diagnostic interviews range from 37% to 56%. We evaluated the effects of gender, age, intellectual abilities and/or behaviour regulation difficulties on the likelihood that a child with WS would be diagnosed with SP. METHODS: A total of 194 6-17 year-olds with WS were evaluated. To best characterise the relations between the predictors and the probability of a SP diagnosis, we explored not only possible linear effects but also curvilinear effects. RESULTS: No gender differences were detected. As age increased, the likelihood of receiving a SP diagnosis decreased. As IQ increased, the probability of receiving a SP diagnosis also decreased. Behaviour regulation difficulties were the strongest predictor of a positive diagnosis. A quadratic relation was detected: The probability of receiving a SP diagnosis gradually rose as behaviour regulation difficulties increased. However, once behaviour regulation difficulties approached the clinical range, the probability of receiving a SP diagnosis asymptoted at a high level. CONCLUSION: Children with behaviour regulation difficulties in or just below the clinical range were at the greatest risk of developing SP. These findings highlight the value of large samples and the importance of evaluating for nonlinear effects to provide accurate model specification when characterising relations among a dependent variable and possible predictors.

Adaptive behavior of 4- through 8-year-old children with Williams syndrome.

The adaptive behavior of forty-one 4- through 8-year-olds with Williams syndrome was assessed using the Vineland Adaptive Behavior Scales-Interview Edition. Based on the cognitive and personality profiles characteristic of children with this syndrome, we predicted that the domains of Socialization and Communication would be relative strengths, whereas Daily Living Skills and Motor Skills would be relative weaknesses. We also expected that Socialization Skills would be more advanced than Communication skills, and that within the Socialization domain, interpersonal skills would be stronger than play/leisure or coping skills. All predictions were confirmed. Adaptive behavior standard score was not related to CA. The children earned similar overall standard scores on the Vineland and the Differential Ability Scales. Interrelations among adaptive behavior, cognitive abilities, and personality characteristics are discussed.

The Williams syndrome cognitive profile.

Williams syndrome is a rare neurodevelopmental disorder caused by a hemizygous deletion of approximately 1.5 megabases on chromosome 7q11.23. In this article, we outline a Williams Syndrome Cognitive Profile (WSCP) that operationalizes the cognitive characteristics of the syndrome using measures of absolute and relative performance on subtests of the Differential Abilities Scales (Elliot, 1990a). Testing confirmed excellent sensitivity and specificity scores for the WSCP. Seventy-four of 84 individuals with Williams syndrome fit the WSCP while only 4 participants in a contrast group met all of the WSCP criteria. It was also found that the WSCP does not vary greatly with chronological age or overall level of cognitive ability for individuals with Williams syndrome. Possible applications for the WSCP include psychoeducational evaluation and empirical research such as the search for genotype/phenotype relations in this genetically based syndrome.

Williams syndrome: cognition, personality, and adaptive behavior.

Williams syndrome is caused by a microdeletion of at least 16 genes on chromosome 7q11.23. The syndrome results in mild to moderate mental retardation or learning disability. The behavioral phenotype for Williams syndrome is characterized by a distinctive cognitive profile and an unusual personality profile. Relative to overall level of intellectual ability, individuals with Williams syndrome typically show a clear strength in auditory rote memory, a strength in language, and an extreme weakness in visuospatial construction. The personality of individuals with Williams syndrome involves high sociability, overfriendliness, and empathy, with an undercurrent of anxiety related to social situations. The adaptive behavior profile for Williams syndrome involves clear strength in socialization skills (especially interpersonal skills related to initiating social interaction), strength in communication, and clear weakness in daily living skills and motor skills, relative to overall level of adaptive behavior functioning. Literature relevant to each of the components of the Williams syndrome behavioral phenotype is reviewed, including operationalizations of the Williams syndrome cognitive profile and the Williams syndrome personality profile. The sensitivity and specificity of these profiles for Williams syndrome, relative to individuals with other syndromes or mental retardation or borderline normal intelligence of unknown etiology, is considered. The adaptive behavior profile is discussed in relation to the cognitive and personality profiles. The importance of operationalizations of crucial components of the behavioral phenotype for the study of genotype/phenotype correlations in Williams syndrome is stressed. MRDD Research Reviews 2000;6:148-158.