Music Affinity and Emotion in Williams Syndrome

2020 ◽  
Vol 12 (2) ◽  
pp. 122
Author(s):  
Philip To Lai
Keyword(s):  
Williams Syndrome ◽  
Cognitive Abilities ◽  
Genetic Disorder ◽  
Cognitive Profile ◽  
Typically Developing ◽  
Spatial Skills ◽  
Moderate Intellectual Disability ◽  
Reasoning Abilities ◽  
Neuropsychological Profile ◽  
Conceptual Reasoning

Williams syndrome (WS) is a neurodevelopmental genetic disorder. Of interest to cognitive scientists is the uneven cognitive profile that is characteristic of the syndrome. In spite of mild to moderate intellectual disability, a complex pattern of strengths and weaknesses is found in their cognitive abilities. Strengths are seen in linguistic abilities and facial processing whereas weaknesses include visuo-spatial skills and conceptual reasoning abilities. Numerous anecdotes exist regarding musical abilities in individuals with WS. To elaborate on the neuropsychological profile of WS and particularly to better understand the affinity to music in individuals with WS, the present study examined the perception and production of music in this population. Results revealed that overall, individuals with WS were more expressive than typically developing individuals when they were involved with music. This study serves to further characterize this unique social and cognitive profile of individuals with WS, adding an affinity to music as a characteristic of their phenotype.

Narrative as a Social Engagement Tool: The Excessive Use of Evaluation in Narratives from Children with Williams Syndrome

2000 ◽  
Vol 10 (2) ◽  
pp. 265-290 ◽  
Author(s):  
Molly Losh ◽  
Ursula Bellugi ◽  
Judy Reilly
Keyword(s):  
Williams Syndrome ◽  
Cognitive Abilities ◽  
Social Engagement ◽  
Picture Book ◽  
Genetic Disorder ◽  
Narrative Development ◽  
Typically Developing ◽  
Complex Syntax ◽  
And Gender ◽  
Matched Comparison

Williams syndrome is a rare genetic disorder characterized by a unique physiological and behavioral profile, involving excessive sociability and relatively spared linguistic abilities in spite of mild to moderate mental retardation. The present study examines the narrative development of children with Williams syndrome and, for the first time, compares their performance to typically developing chronological-age matched children to examine the development of both structural linguistic abilities as well as the use of evaluation to elaborate and enrich narrative. Thirty children with Williams syndrome (5- through 10-years-old) and 30 typically developing age- and gender-matched comparison children were asked to tell a story from a wordless picture book. Results indicated that as a group, children with Williams syndrome committed significantly more morphological errors and used less complex syntax than comparison children, not surprising considering their language delay and impaired cognitive abilities. Significantly, children with Williams syndrome greatly exceeded comparison children in their elaboration and use of evaluative devices and showed particular preference for types of evaluation which serve as social engagement devices, reflecting their profile of excessive sociability. (Williams syndrome, Narrative, Evaluation)

scholarly journals Understanding Number Line Estimation in Williams Syndrome and Down Syndrome

2019 ◽  
Vol 50 (2) ◽  
pp. 583-591 ◽  
Author(s):  
V. Simms ◽  
A. Karmiloff-Smith ◽  
E. Ranzato ◽  
J. Van Herwegen
Keyword(s):  
Down Syndrome ◽  
Williams Syndrome ◽  
Neurodevelopmental Disorders ◽  
Number Line ◽  
Mental Number Line ◽  
Typically Developing ◽  
Verbal Intelligence ◽  
Spatial Skills ◽  
Number Line Estimation ◽  
Group Comparisons

Abstract Previous studies suggest that tasks dependent on the mental number line may be difficult for Williams Syndrome (WS) and Down Syndrome (DS) groups. However, few have directly assessed number line estimation in these groups. The current study assessed 28 WS, 25 DS and 25 typically developing (TD) participants in non-verbal intelligence, number familiarity, visuo-spatial skills and number line estimation. Group comparisons indicated no differences in number line estimation. However, the WS group displayed difficulties with visuo-spatial skills and the DS group displayed difficulties with number familiarity. Differential relationships between number line estimation and visuo-spatial/number familiarity skills were observed across groups. Data is discussed in the context of assessment of skills in neurodevelopmental disorders.

Diurnal Cortisol Profile in Williams Syndrome in Novel and Familiar Settings

2013 ◽  
Vol 118 (3) ◽  
pp. 201-210 ◽  
Author(s):  
Miriam Diane Lense ◽  
Andrew J. Tomarken ◽  
Elisabeth M. Dykens
Keyword(s):  
Stress Response ◽  
Williams Syndrome ◽  
Social Issues ◽  
Genetic Disorder ◽  
Somatic Complaints ◽  
Cortisol Awakening Response ◽  
The Novel ◽  
Typically Developing ◽  
Diurnal Cortisol ◽  
Social Difficulties

Abstract Williams syndrome (WS) is a neurodevelopmental genetic disorder associated with high rates of anxiety and social issues. We examined diurnal cortisol, a biomarker of the stress response, in adults with WS in novel and familiar settings, and compared these profiles to typically developing (TD) adults. WS and TD participants had similar profiles in a familiar setting, while participants with WS had elevated cortisol late in the day in the novel setting when social demands were higher. The cortisol awakening response in WS was associated with parent-reported levels of somatic complaints and social difficulties. Results suggest that adults with WS have a typical diurnal cortisol profile that may be sensitive to social and activity transitions throughout the day.

Rhythmic Abilities of Adolescents and Adults with Williams Syndrome

2011 ◽  
Vol 29 (1) ◽  
pp. 79-91 ◽  
Author(s):  
Pastora Martínez-Castilla ◽  
María Sotillo ◽  
Ruth Campos
Keyword(s):  
Williams Syndrome ◽  
Cognitive Deficits ◽  
Discrimination Task ◽  
Cognitive Profile ◽  
Chronological Age ◽  
Control Group ◽  
Typically Developing ◽  
Reproduction Task ◽  
Adolescents And Adults ◽  
Rhythmic Performance

although rhythmic abilities have often been described as strengths within the cognitive profile of individuals with Williams syndrome (WS), the literature in this respect is limited and has offered inconsistent results. In this study, 20 adolescents and adults with WS and a control group of 40 typically developing individuals matched for chronological age were presented with a rhythmic patterns discrimination task and a rhythmic patterns reproduction task. Individuals with WS performed significantly lower than their control peers in both tasks. In addition, rhythmic impairments in WS were explained by the cognitive deficits that are characteristic of the syndrome. These results suggest that rhythmic performance in individuals with WS is affected by their cognitive deficits and that rhythmic skills in WS are not independent of general cognition.

I. The Neurocognitive Profile of Williams Syndrome: A Complex Pattern of Strengths and Weaknesses

2000 ◽  
Vol 12 (supplement 1) ◽  
pp. 7-29 ◽  
Author(s):  
Ursula Bellugi ◽  
Liz Lichtenberger ◽  
Wendy Jones ◽  
Zona Lai ◽  
Marie St. George
Keyword(s):  
Williams Syndrome ◽  
Cognitive Abilities ◽  
Interdisciplinary Research ◽  
Genetic Basis ◽  
Cognitive Profile ◽  
Cognitive Domains ◽  
Cognitive Characteristics ◽  
Neurocognitive Profile ◽  
Moderate Range ◽  
The Brain

The rare, genetically based disorder, Williams syndrome (WMS), produces a constellation of distinctive cognitive, neuroanatomical, and electrophysiological features which we explore through the series of studies reported here. In this paper, we focus primarily on the cognitive characteristics of WMS and begin to forge links among these characteristics, the brain, and the genetic basis of the disorder. The distinctive cognitive profile of individuals with WMS includes relative strengths in language and facial processing and profound impairment in spatial cognition. The cognitive profile of abilities, including what is ‘typical’ for individuals with WMS is discussed, but we also highlight areas of variability across the group of individuals with WMS that we have studied. Although the overall cognitive abilities (IQs) of individuals with WMS are typically in the mild-to-moderate range of mental retardation, the peaks and valleys within different cognitive domains make this syndrome especially intriguing to study across levels. Understanding the brain basis (and ultimately the genetic basis) for higher cognitive functioning is the goal we have begun to undertake with this line of interdisciplinary research.

Williams Syndrome

2010 ◽  
Author(s):  
Judy Reilly ◽  
Philip Lai
Keyword(s):  
Williams Syndrome ◽  
Language Skills ◽  
Genetic Disorder ◽  
Therapeutic Interventions ◽  
Chromosome Band ◽  
Multiple Perspectives ◽  
Neuropsychological Profile ◽  
The Face ◽  
Impaired Cognition ◽  
Elastic Protein

Williams syndrome (WS) is a rare neurodevelopmental genetic disorder stemming from a hemizygous deletion of about 20–28 genes from chromosome band 7.11.23q (Ewart et al. 1993) including the gene for elastin. Early estimates of incidence proposed 1 in 20,000 live births (Greenberg 1989), but current estimates are 1 in 7,500 (Stromme, Bjornstad, and Ramstad 2002). Williams syndrome was first recognized by a pediatrician, Dr. JCP Williams in 1961, who described a group of children with “elfin facies” and supravalvular aortic stenosis (Williams, Barratt-Boyes, and Lowe 1961). Shortly thereafter, Dr. Alois Beuren (1962) identified another group of children with similar features, including an intolerance to calcium. As such, this syndrome has also been called Williams-Beuren syndrome or infantile hypercalcemia. The early descriptions of children and adolescents with WS noted a set of characteristic facial and behavioral features: “an unusual command of language combined with an unexpectedly polite, gentle and open manner” (von Armin and Engel 1964). Twenty years later, WS caught the interest of cognitive scientists when it was seen as a possible case of spared language in the face of impaired cognition (Bellugi, Sabo, and Vaid 1988; Bellugi, Wang, and Jernigan 1994). The early studies noted that, in spite of IQs ranging from 40–70, with an average full-scale IQ of 55 (Bellugi et al. 2001) and significantly impaired visuospatial cognition, adolescents with WS had excellent language skills (Bellugi, Lai, and Wang 1997; Reilly, Klima, and Bellugi 1990). In the ensuing 20 years, recognizing WS as a unique opportunity to investigate genetic influences on brain development and cognitive and social processes, investigators have approached the puzzles of WS from multiple perspectives. In this chapter, we provide an overview of WS, focusing on its cognitive and neuropsychological profile from a developmental perspective and where known, the possible neural and genetic underpinnings of the WS profile. We close with a brief discussion of therapeutic interventions for WS. The WS deletion invariably includes the gene for elastin (ELN), which codes for an elastic protein in connective tissue that is abundant in large blood vessels such as the aorta (Lowery et al. 1995).

scholarly journals Learning and Using Abstract Words: Evidence from Clinical Populations

2017 ◽  
Vol 2017 ◽  
pp. 1-8 ◽  
Author(s):  
Maria Luisa Lorusso ◽  
Michele Burigo ◽  
Alessandro Tavano ◽  
Anna Milani ◽  
Sara Martelli ◽  
...  
Keyword(s):  
Down Syndrome ◽  
Williams Syndrome ◽  
Cognitive Profile ◽  
Typically Developing ◽  
Abstract Concepts ◽  
Visual Spatial ◽  
Abstract Words ◽  
Concrete Concepts ◽  
Concrete Words

It has been shown that abstract concepts are more difficult to process and are acquired later than concrete concepts. We analysed the percentage of concrete words in the narrative lexicon of individuals with Williams Syndrome (WS) as compared to individuals with Down Syndrome (DS) and typically developing (TD) peers. The cognitive profile of WS is characterized by visual-spatial difficulties, while DS presents with predominant impairments in linguistic abilities. We predicted that if linguistic abilities are crucial to the development and use of an abstract vocabulary, DS participants should display a higher concreteness index than both Williams Syndrome and typically developing individuals. Results confirm this prediction, thus supporting the hypothesis of a crucial role of linguistic processes in abstract language acquisition. Correlation analyses suggest that a maturational link exists between the level of abstractness in narrative production and syntactic comprehension.

Genetic Developmental Disorders and Numerical Competence across the Lifespan

2014 ◽  
Author(s):  
Jo Van Herwegen ◽  
Annette Karmiloff-Smith
Keyword(s):  
Cognitive Abilities ◽  
Developmental Disorders ◽  
Genetic Disorder ◽  
Typically Developing ◽  
Cognitive Profiles ◽  
Numerical Competence ◽  
Developmental Approach ◽  
Attention Systems ◽  
Basic Level ◽  
Approximate Magnitude

Due to their frequent uneven cognitive profiles, genetic developmental disorders allow researchers to investigate which numerical sub-system of those present in typically developing infants best predicts subsequent numerical abilities. More importantly, they can provide evidence of which other cognitive abilities outside number are necessary for the successful development of these numerical sub-systems. We discuss evidence from cross-syndrome comparisons of adults, adolescents, children, and infants with Williams syndrome and those with Down syndrome to show that the approximate magnitude sub-system is crucial for later number development. In addition, we show that specific problems outside the number domain, and within basic-level visual and attention systems contribute to an explanation of the difficulties and proficiencies observed within each genetic disorder. Finally, we argue that a truly developmental approach is critical when using the cross-syndrome design in order to reveal subtle differences that impact over time on the development of cognitive abilities.

scholarly journals Williams syndrome: Reduced attention to other’s eyes in a hypersocial phenotype

2020 ◽  
Author(s):  
Johan Lundin Kleberg ◽  
Deborah Riby ◽  
Christine Fawcett ◽  
Hanna Björlin Avdic ◽  
Matilda A. Frick ◽  
...  
Keyword(s):  
Intellectual Disability ◽  
Social Interaction ◽  
Williams Syndrome ◽  
Genetic Disorder ◽  
Age Groups ◽  
Social Motivation ◽  
Social Understanding ◽  
Typically Developing ◽  
Gaze Behavior ◽  
Saccadic Velocity

Williams syndrome (WS) is a rare genetic disorder which leads to high social motivation as well as intellectual disability and difficulties with social interaction. Attention to others’ eyes is crucial for social understanding. Individuals with WS are typically highly attentive to faces, but there is a lack of knowledge about how they attend to other’s eyes, and the mechanisms underlying potential alterations in behavior. It has been suggested that physiological hypo-arousal enables individuals with WS to maintain their gaze longer at other’s faces. The aim of this study was to better understand if there is atypical gaze behavior among individuals with WS which might underlie their reduced social understanding and related difficulties with social interaction. We examined the speed and likelihood of gaze shifts to and from other’s eyes in individuals with WS (n = 37; mean age 23 years), and controls (n = 167) in stratified age groups (7 months, 8-12 years, 13-17 years, adults). Peak saccadic velocity was studied as an index of arousal. Individuals with WS were less likely, and slower, to orient to the eyes compared with typically developing controls in all age groups from eight years of age but did not differ from 7-month-old infants. Peak saccadic velocity was reduced in WS, replicating previous results of hypo-arousal. The results were supported by both frequentist and Bayesian statistics. Despite the hyper-social behavioral phenotype, WS is associated with reduced attention to others’ eyes. This could contribute to the difficulties with complex social interaction observed in WS.

Probed Serial Recall in Williams Syndrome

2005 ◽  
Vol 48 (2) ◽  
pp. 360-371 ◽  
Author(s):  
Jon Brock ◽  
Teresa McCormack ◽  
Jill Boucher
Keyword(s):  
Serial Recall ◽  
Williams Syndrome ◽  
Short Term Memory ◽  
Genetic Disorder ◽  
Serial Order ◽  
Typically Developing ◽  
Control Groups ◽  
Typically Developing Children ◽  
Short Term ◽  
Lexical Knowledge

Williams syndrome is a genetic disorder that, it has been claimed, results in an unusual pattern of linguistic strengths and weaknesses. The current study investigated the hypothesis that there is a reduced influence of lexical knowledge on phonological short-term memory in Williams syndrome. Fourteen children with Williams syndrome and 2 vocabulary-matched control groups, 20 typically developing children and 13 children with learning difficulties, were tested on 2 probed serial-recall tasks. On the basis of previous findings, it was predicted that children with Williams syndrome would demonstrate (a) a reduced effect of lexicality on the recall of list items, (b) relatively poorer recall of list items compared with recall of serial order, and (c) a reduced tendency to produce lexicalization errors in the recall of nonwords. In fact, none of these predictions were supported. Alternative explanations for previous findings and implications for accounts of language development in Williams syndrome are discussed.

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