Behavioral features of Williams Beuren syndrome compared to Fragile X syndrome and subjects with intellectual disability without defined etiology

• Published in: Research in developmental disabilities Vol. 32; no. 2; pp. 643 - 652
• Main Authors: Pérez-García, D., Granero, R., Gallastegui, F., Pérez-Jurado, L.A., Brun-Gasca, C.
• Format: Journal Article
• Language: English
• Published: Amsterdam Elsevier Ltd 01.03.2011; Elsevier
• Subjects: Adolescent; Adult; Adult and adolescent clinical studies; Aetiology; Aggression; Anxiety; Anxiety Disorders - etiology; Anxiety Disorders - psychology; Anxiety-Depression; Behavior Problems; Behavioral phenotype; Biological and medical sciences; Check Lists; Child; Child Behavior; Child Behavior Checklist; Child Behavior Disorders - etiology; Child Behavior Disorders - psychology; Child clinical studies; Child, Preschool; Chromosome aberrations; Chromosome fragility (bloom syndrome, ataxia telangiectasia, fanconi anemia, x-linked mental retardation...); Cognitive Processes; Delinquency; Depression (Psychology); Depressive Disorder - etiology; Depressive Disorder - psychology; Developmental Disabilities; Developmental disorders; Etiology; Female; Fragile X syndrome; Fragile X Syndrome - complications; Fragile X Syndrome - psychology; Gender Differences; Genetic Disorders; Humans; Intellectual deficiency; Intellectual disability; Intellectual Disability - complications; Intellectual Disability - etiology; Intellectual Disability - psychology; Intelligence; Intelligence Quotient; Learning disabilities; Male; Medical genetics; Medical sciences; Mental Retardation; Neurological Impairments; Phenotype; Phenotypes; Psychology. Psychoanalysis. Psychiatry; Psychopathology; Psychopathology. Psychiatry; Symptoms (Individual Disorders); Williams Syndrome - complications; Williams Syndrome - genetics; Williams Syndrome - psychology; Williams-Beuren syndrome; Young Adult; IQ; Anxiety; Intellectual disability; Fragile X syndrome; Williams-Beuren syndrome; Behavioral phenotype; Chromosomal aberration; Chromosome fragility; Anxiety disorder; Cognition; Developmental disorder; Mental retardation; Behavioral analysis; Etiology; Intellectual ability; Child; Human; Preschool age; Handicap; Williams syndrome; Phenotype; School age; Adolescent; Young adult; Intellectual deficiency; Intelligence quotient
• ISSN: 0891-4222; 1873-3379
• DOI: 10.1016/j.ridd.2010.12.005

▶ Definition the psychopathological profile of WBS in comparison to FXS and IDNS. ▶ High prevalence of anxiety, depression and attention problems in WBS. ▶ Delinquent and aggressive behaviors and externalizing problems are rare in WBS patients. ▶ Behavioral problems are secondary to the intellectual disability and not specific of the syndrome. ▶ Associations between IQ and anxiety items. Williams-Beuren syndrome (WBS) is a genetically determined neurodevelopmental disorder caused by a heterozygous deletion of 26–28 genes on chromosome band 7q11.23. During the past few years, researchers and clinicians have significantly contributed to define the phenotype of the syndrome, including its cognitive and behavioral aspects. However, it is not well known yet whether the psychological problems are specific to the syndrome or secondary to the intellectual disability (ID). The aim of our study was to better define the psychopathological profile of WBS and whether or not it is related with IQ or anxiety symptoms. Twenty-five subjects (12 girls, 13 boys) with a diagnosis of WBS were compared to 27 boys with Fragile X Syndrome and to 24 boys with ID of non-specific etiology using the Child Behavior Checklist. Anxiety, depression and attention problems were the main behavioral problems found in WBS with no gender differences. Significant differences between cohorts were observed in somatic complaints, delinquent behavior, aggressive behavior, and externalizing problems. Some associations between IQ and anxiety items were found. The findings are discussed in terms of behavioral phenotypes, genetic implications and ID.