Cognitive abilities in Williams syndrome

Miezah, Daniel; Porter, Melanie; Batchelor, Jennifer; Boulton, Kelsie A; Veloso, Gabrielle Campos

doi:10.1016/j.ridd.2020.103701

Cited by 14 publications

(8 citation statements)

References 54 publications

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“…This shows that there are some children with WS that have few sensory processing difficulties across development. It is possible the children in this subgroup have greater executive functioning overall and subsequent capacity to process sensory information at both T1 and T2 (Miezah et al, 2020 ). The high-low group had high impairments at T1 and low impairments at T2.…”

Section: Discussionmentioning

confidence: 99%

“…Individuals with WS tend to show uneven cognitive and behavioural patterns and there is wide individual variability within the WS population (Miezah et al, 2020 ; Porter & Coltheart, 2005 ; Van Herwegen et al, 2011 ) including for sensory profiles. John and Mervis ( 2010 ) found that participants’ scores on the SSP subscales clustered into two homogenous subgroups of sensory processing ability: high impairment ( n = 31) and low impairment ( n = 40).…”

Section: Individual Differences In Williams Syndromementioning

confidence: 99%

“…The cognitive profile of individuals with WS is typically uneven, with better language and face recognition skills in contrast to poorer planning and visuo-spatial abilities. In addition, individuals with WS tend to have mild to moderate intellectual difficulties with an average IQ of 55, although, there is much variability in cognitive ability (Miezah et al, 2020 ). Behaviourally, WS is characterised by hyper-sociability; an intense drive to form bonds with others and little fear of strangers (Shore et al, 2017 ).…”

Section: Introductionmentioning

confidence: 99%

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Sensory Processing in Williams Syndrome: Individual differences and changes over time

Powell¹,

Herwegen²

2021

J Autism Dev Disord

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This study examined individual differences as well as the development of sensory processing difficulties in children with Williams syndrome (WS) using a cross-sectional (Experiment 1) and longitudinal design (Experiment 2). In Experiment 1, a clustering approach of sensory processing scores suggested two groups. Experiment 2 showed that the clusters identified in Experiment 1 were not stable across development, especially for those with high sensory impairments at either time point. Yet, most children experienced high impairments in sensory registration at both time points, suggesting impaired registration is a core phenotype of sensory processing in children with WS across development. Possible mechanisms, limitations and implications are discussed.

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Section: Discussionmentioning

confidence: 99%

Section: Individual Differences In Williams Syndromementioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Sensory Processing in Williams Syndrome: Individual differences and changes over time

Powell¹,

Herwegen²

2021

J Autism Dev Disord

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“…In addition, in subjects with WS you can see a good memory of stimuli in the face of difficulties in other memory skills (Kaplan, Wang, & Francke, 2001) and a dissociation between face processing, language and social cognition (Bellugi, Bihrle, Jernigan, Trauner, & Doherty, 1990;Udwin, Yule, & Martin, 1987). Chronological age did not relate significantly to cognitive abilities (Miezah, Porter, Batchelor, Boulton, & Veloso, 2020).…”

Section: Introductionmentioning

confidence: 92%

Analysis of Receptive Vocabulary Development in Williams Syndrome

Sepúlveda¹,

López²

2020

OJML

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Although there is some consensus when it comes to establishing the lexicon as one of the strengths of language in people with Williams Syndrome (WS), little is known about its evolution throughout development and changes based on age. The objective of this study was to find out if there are differences in receptive vocabulary between childhood and adolescence and into adulthood. In this research, 19 people with WS between 6 and 20 years old, divided into two age ranges (6-11; 12-20) and matched in mental age, were evaluated through the Peabody Vocabulary Test. The results show significant differences between both groups in favor of the group with the oldest chronological age and a direct correlation between chronological age and receptive vocabulary development, regardless of mental age. These data support the natural evolution of the passive lexicon in people with WS.

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“…Several studies have identified specific deficits in executive functioning (working memory, attentional abilities, and inhibition), problem-solving, and visuospatial skills ( Camp et al, 2016 ; Heiz and Barisnikov, 2016 ; D’Souza et al, 2020 ). In contrast, auditory processing and face recognition are strengths in the WS profile ( D’Souza et al, 2015 ; Miezah et al, 2020 ). Akin to the uneven cognitive profile, they also appear to show relative strengths and weaknesses in the motor profile, in the context of persisting fine and gross motor difficulties into childhood and adulthood ( Mayall et al, 2021 ).…”

Section: Introductionmentioning

confidence: 99%

Late phonological development in Williams syndrome

Pérez

Martínez²,

Diez-Itza³

2022

Front. Psychol.

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Williams syndrome is a neurodevelopmental genetic disorder characterized by a unique phenotype, including mild to moderate intellectual disability and an uneven neuropsychological profile of relative strengths and weaknesses. Language structure components (i.e., phonology, morphosyntax, and vocabulary) have been considered an area of specific ability compared to pragmatic language use. However, research on phonological development in Williams syndrome is very scarce, and it suggests atypical patterns. Therefore, the aim of the present study was to explore the profiles of late phonological development in Spanish-speaking children, adolescents, and adults with Williams syndrome, based on the analysis of five classes of processes (Syllable Structure, Substitution, Omission, Assimilation, and Addition) in spontaneous speech. The phonological profiles of seven children (aged 3–8 years), and seven adolescents and young adults (aged 14–25 years) with Williams syndrome were compared with two normative groups of typically developing (TD) children at different stages of late phonological development (aged 3 and 5 years). The frequency of phonological processes in the group of children with Williams syndrome was similar to that of 3-year-old TD children, which suggests that they would be in the first stage of late phonological development (expansion stage). The group of older individuals with Williams syndrome showed a much lower frequency of processes, similar to that of 5-year-old TD children in the last stage of phonological development (resolution stage). However, their phonological processes appeared to be persistent and independent of chronological age. Furthermore, asynchronies in quantitative and qualitative profiles (relative frequency) indicated atypical and complex trajectories in late phonological development, which cannot be described as simply delayed or protracted. Remarkable individual differences were observed, especially in the group of adolescents and adults with Williams syndrome, although the majority of cases conformed to the modal profiles of their groups. A major tendency for Omission, including final consonant deletion, may be considered atypical and specific to Williams syndrome at all ages. The results of the present study raise the need for continued and appropriate phonological assessment and treatment for people with Williams syndrome across the lifespan.

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Cognitive abilities in Williams syndrome

Cited by 14 publications

References 54 publications

Sensory Processing in Williams Syndrome: Individual differences and changes over time

Sensory Processing in Williams Syndrome: Individual differences and changes over time

Analysis of Receptive Vocabulary Development in Williams Syndrome

Late phonological development in Williams syndrome

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