Refining Behavioral Phenotypes: Personality—Motivation in Williams and Prader-Willi Syndromes

Dykens, Elisabeth M.; Rosner, Beth

doi:10.1352/0895-8017(1999)104<0158:rbppiw>2.0.co;2

Cited by 139 publications

(86 citation statements)

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“…They are no better than wellmatched controls with LID in on-line tasks that tap the ability to decode social-affective information, just as they are no better in socialcognitive tasks (Tager-Flusberg & Sullivan, 2000). Nevertheless, children and adults with WMS appear to have a unique social phenotype characterised by unusual friendliness, especially toward strangers, and a warm empathic manner in engaging with others (Dykens & Rosner, 1999;Gosch & Pankau, 1994;Jones et al, 2000). It remains to be seen whether this phenotype is best interpreted in terms of personality variables (cf.…”

Section: Discussionmentioning

confidence: 99%

Social-perceptual abilities in adolescents and adults with Williams syndrome

Skwerer

Verbalis

Schofield

et al. 2006

Cognitive Neuropsychology

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People with Williams syndrome (WMS) have a unique social phenotype characterised by unusually strong interest in other people and an engaging and empathic personality. Two experiments were designed to test whether this phenotype is associated with relatively spared abilities to decode mental-state information from nonverbal cues. The first experiment involved a modified version of the revised Eyes Test. The second experiment probed the ability to label emotions from brief dynamic facial displays. Adolescents and adults with WMS were compared to age-, IQ-, and language-matched participants with learning/intellectual disabilities, and age-matched normal controls. In both experiments the WMS group performed at a significantly lower level than the normal controls, and no different from the well-matched comparison-group with intellectual disabilities. These findings, contradicting earlier reports in the literature, argue against the view that in WMS social-perceptual abilities are relatively spared and can explain the social profile associated with this neurodevelopmental disorder.

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Section: Discussionmentioning

confidence: 99%

Social-perceptual abilities in adolescents and adults with Williams syndrome

Skwerer

Verbalis

Schofield

et al. 2006

Cognitive Neuropsychology

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“…It has a prevalence of 1: 7500 to 20000 live births (Morris, Demsey, Leonard, Dilts, & Blackburn, 1988;Strømme, Bjørnstad, & Ramstad, 2002) and is characterized by mild to severe intellectual disability and a defined cognitive profile (Mervis et al, 2000). Individuals with WS are often described as very sociable, empathic and friendly (Dykens & Rosner, 1999;Gosh & Pankau, 1994, but also present high levels of anxiety and fears (Dykens, 2003;Leyfer, Woodruff-Borden, KleinTasman, Fricke, & Mervis, 2006), difficulties when interacting with others (Frigerio et al, 2006), poor social relationships (Davies, Udwin, & Howlin, 1998) and indiscriminate approach to strangers (Jones et al, 2000).…”

Section: Recognition Of Emotional Facial Expressions In Williams Syndmentioning

confidence: 99%

Facial emotion recognition in Williams syndrome and Down syndrome: A matching and developmental study

Martínez‐Castilla

Burt

Borgatti

et al. 2014

Child Neuropsychology

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Citation for published item:wrt¡ %nezEgstillD F nd furtD wF nd forgttiD F nd qglirdiD gF @PHISA 9pil emotion reognition in illims syndrome nd hown syndrome X mthing nd developmentl studyF9D ghild neuropsyhologyFD PI @SAF ppF TTVETWPF Further information on publisher's website: Additional information:Use policyThe full-text may be used and/or reproduced, and given to third parties in any format or medium, without prior permission or charge, for personal research or study, educational, or not-for-prot purposes provided that:• a full bibliographic reference is made to the original source • a link is made to the metadata record in DRO • the full-text is not changed in any way The full-text must not be sold in any format or medium without the formal permission of the copyright holders.Please consult the full DRO policy for further details. In this study both the matching and developmental trajectories approaches were used to clarify the development of facial expression recognition in Williams syndrome (WS) and Down syndrome (DS). The matching approach showed that neither individuals with WS nor DS exhibit a specific proficiency for the expression of happiness or a specific impairments for negative emotions but presented the same pattern of emotion recognition as typically developing (TD) individuals. Thus, the better performance on the recognition of positive compared to negative emotions often reported in WS and DS is not specific to these populations but represents a typical pattern. Prior studies based on the matching approach suggested that the development of facial expression recognition is delayed in WS and atypical in DS. Nevertheless, and even though performance levels were lower in DS than in WS, the developmental trajectories approach used in this study found that not only individuals with DS but also those with WS present atypical developmental trajectories. Unlike in the TD participants where developmental changes were observed along with age, in the WS and DS development was static. Thus, both individuals with WS and those with DS reach early maximum levels of facial expression recognition due to cognitive constraints.

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“…Second, two or more syndromes may be similar in certain ways, but different in others. Like children with Down syndrome, then, children with Williams syndrome are also sociable (albeit in more disinhibited ways; Dykens & Rosner, 1999) while also showing higher rates of anxiety and fears (Dykens, 2003). Given the many different behavioral patterns common to different genetic disorders, it may be possible to tease apart the effects of specific behaviors on particular parental reactions or behaviors.…”

Section: Reacting To Etiology-related Personalities and Dependency Cuesmentioning

confidence: 99%

Studying Interactions, Reactions, and Perceptions: Can Genetic Disorders Serve as Behavioral Proxies?

Hodapp

2004

J Autism Dev Disord

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Different genetic disorders predispose individuals to display specific, etiology-related profiles, personalities, and maladaptive behaviors. Using groups with genetic etiologies as stand-ins or proxies for a specific behavior or set of behaviors, one can then examine how others in the child's environment react and whether such reactions are limited to a particular disorder or occur generally to all individuals showing that behavior. Just as twins, adopted, and institutionalized children have all been used as natural experiments to tell us about typical development, so too can groups with specific genetic syndromes help us to understand the nature and mechanisms of the reactions and behaviors of others.

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Refining Behavioral Phenotypes: Personality—Motivation in Williams and Prader-Willi Syndromes

Cited by 139 publications

References 0 publications

Social-perceptual abilities in adolescents and adults with Williams syndrome

Social-perceptual abilities in adolescents and adults with Williams syndrome

Facial emotion recognition in Williams syndrome and Down syndrome: A matching and developmental study

Studying Interactions, Reactions, and Perceptions: Can Genetic Disorders Serve as Behavioral Proxies?

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