Characterizing associations and dissociations between anxiety, social, and cognitive phenotypes of Williams syndrome

• Published in: Research in developmental disabilities 2014-10, Vol.35 (10), p.2403-2415
• Main Authors: Ng, Rowena, Järvinen, Anna, Bellugi, Ursula
• Format: Article
• Language: English
• Subjects: Adaptation, Psychological; Adolescent; Adult; Adult and adolescent clinical studies; Anxiety; Anxiety - psychology; Anxiety Disorders - psychology; Biological and medical sciences; Case-Control Studies; Chromosome aberrations; Cognition; Cognitive abilities; Emotional wellbeing; Female; Humans; Insight; Intellectual deficiency; Intellectual Disability - psychology; Intellectual impairment; Maladaptive behaviors; Male; Medical genetics; Medical sciences; Middle Aged; Neurodevelopmental disorder; Phenotype; Phenotypes; Psychology. Psychoanalysis. Psychiatry; Psychopathology. Psychiatry; Social Behavior; Symptoms; Williams syndrome; Williams Syndrome - psychology; Young Adult
• ISSN: 0891-4222; 1873-3379
• DOI: 10.1016/j.ridd.2014.06.010

•Anxiety is a core feature of the Williams syndrome (WS) phenotype.•The study examined the specific nature of anxieties in WS contrasted with typical development (TD).•Associations between cognitive/social functioning and anxiety were also examined.•Increased physiological and subjective anxieties characterized individuals with WS.•Increased cognitive but not social functioning was associated with anxiety only in WS. Williams syndrome (WS) is a neurogenetic disorder known for its “hypersocial” phenotype and a complex profile of anxieties. The anxieties are poorly understood specifically in relation to the social-emotional and cognitive profiles. To address this gap, we employed a Wechsler intelligence test, the Brief Symptom Inventory, Beck Anxiety Inventory, and Salk Institute Sociability Questionnaire, to (1) examine how anxiety symptoms distinguish individuals with WS from typically developing (TD) individuals; and (2) assess the associations between three key phenotypic features of WS: intellectual impairment, social-emotional functioning, and anxiety. The results highlighted intensified neurophysiological symptoms and subjective experiences of anxiety in WS. Moreover, whereas higher cognitive ability was positively associated with anxiety in WS, the opposite pattern characterized the TD individuals. This study provides novel insight into how the three core phenotypic features associate/dissociate in WS, specifically in terms of the contribution of cognitive and emotional functioning to anxiety symptoms.