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Distinctive personality and behavior...

Klein-Tasman, Bonita Penny.

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Distinctive personality and behavioral characteristics of 8-, 9-, and 10-year-old children with Williams syndrome.

Record Type:	Electronic resources : Monograph/item
Title/Author:	Distinctive personality and behavioral characteristics of 8-, 9-, and 10-year-old children with Williams syndrome./
Author:	Klein-Tasman, Bonita Penny.
Description:	126 p.
Notes:	Source: Dissertation Abstracts International, Volume: 61-08, Section: B, page: 4464.
Contained By:	Dissertation Abstracts International61-08B.
Subject:	Psychology, Developmental. -
Online resource:	http://pqdd.sinica.edu.tw/twdaoapp/servlet/advanced?query=9982471
ISBN:	059988990X

Distinctive personality and behavioral characteristics of 8-, 9-, and 10-year-old children with Williams syndrome.
Klein-Tasman, Bonita Penny.

Distinctive personality and behavioral characteristics of 8-, 9-, and 10-year-old children with Williams syndrome. - 126 p.

Source: Dissertation Abstracts International, Volume: 61-08, Section: B, page: 4464.

Thesis (Ph.D.)--Emory University, 2000.

Williams syndrome is a multifaceted disorder resulting from a hemizygous microdeletion of chromosome 7q11.23 spanning at least 16 genes. Phenotypic descriptions in several domains (physiology and natural history, cognitive abilities) are relatively well-defined. Previous research has indicated that individuals with Williams syndrome have a distinctive personality, but an empirically derived personality profile has not been developed. The objective of the current investigation was to develop a personality profile that is descriptive of and distinctive to children with Williams syndrome. Participants were 23 8- to 10-year-old children with Williams syndrome and 20 8- to 10-year-old children with developmental disabilities of other etiologies. Participant groups had equivalent intellectual abilities. Parents completed measures of childhood temperament (Children's Behavior Questionnaire; CBQ), personality (parent report, short form of Multidimensional Personality Questionnaire; MPQ), fearfulness (Fear Survey Schedule for Children - Revised; FSSC-R), and problem behaviors (Child Behavior Checklist; CBCL). Using group comparisons and signal detection theory, the personality characteristics of children with Williams syndrome and children with developmental disabilities of other etiologies were contrasted. On the CBQ, high mean ratings on Shyness (reverse coded) and Empathy together characterized 96% of the children in the Williams Syndrome group, and only 15% of the Mixed Etiology group. On the MPQ, high ratings on items measuring certain characteristics combined (Gregarious, People-Oriented, Tense, Sensitive, and Visible) were characteristic of 96% of the Williams Syndrome group and only 15% of the Mixed Etiology group. Given the effectiveness of these measures at capturing the distinctive personality of the children with Williams syndrome in this study, the CBQ and MPQ are promising tools for extension of the personality profile observed to younger and older individuals with Williams syndrome, respectively. No group differences in problem behaviors were observed. Across measures, the Williams syndrome group showed significantly higher levels of anxiety than the Mixed Etiology group, but there were no group differences in fearfulness. Results are related to current findings in the literature about temperament and personality of individuals with Williams syndrome, to current models of personality, and to current theorizing regarding the conceptual distinction between fear and anxiety. The personality profiles emerging from the CBQ and MPQ provide a crucial step toward investigations of genotype/phenotype relations.

ISBN: 059988990XSubjects--Topical Terms:

1017557
Psychology, Developmental.

Distinctive personality and behavioral characteristics of 8-, 9-, and 10-year-old children with Williams syndrome.
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520 $a Williams syndrome is a multifaceted disorder resulting from a hemizygous microdeletion of chromosome 7q11.23 spanning at least 16 genes. Phenotypic descriptions in several domains (physiology and natural history, cognitive abilities) are relatively well-defined. Previous research has indicated that individuals with Williams syndrome have a distinctive personality, but an empirically derived personality profile has not been developed. The objective of the current investigation was to develop a personality profile that is descriptive of and distinctive to children with Williams syndrome. Participants were 23 8- to 10-year-old children with Williams syndrome and 20 8- to 10-year-old children with developmental disabilities of other etiologies. Participant groups had equivalent intellectual abilities. Parents completed measures of childhood temperament (Children's Behavior Questionnaire; CBQ), personality (parent report, short form of Multidimensional Personality Questionnaire; MPQ), fearfulness (Fear Survey Schedule for Children - Revised; FSSC-R), and problem behaviors (Child Behavior Checklist; CBCL). Using group comparisons and signal detection theory, the personality characteristics of children with Williams syndrome and children with developmental disabilities of other etiologies were contrasted. On the CBQ, high mean ratings on Shyness (reverse coded) and Empathy together characterized 96% of the children in the Williams Syndrome group, and only 15% of the Mixed Etiology group. On the MPQ, high ratings on items measuring certain characteristics combined (Gregarious, People-Oriented, Tense, Sensitive, and Visible) were characteristic of 96% of the Williams Syndrome group and only 15% of the Mixed Etiology group. Given the effectiveness of these measures at capturing the distinctive personality of the children with Williams syndrome in this study, the CBQ and MPQ are promising tools for extension of the personality profile observed to younger and older individuals with Williams syndrome, respectively. No group differences in problem behaviors were observed. Across measures, the Williams syndrome group showed significantly higher levels of anxiety than the Mixed Etiology group, but there were no group differences in fearfulness. Results are related to current findings in the literature about temperament and personality of individuals with Williams syndrome, to current models of personality, and to current theorizing regarding the conceptual distinction between fear and anxiety. The personality profiles emerging from the CBQ and MPQ provide a crucial step toward investigations of genotype/phenotype relations.
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