Article
Multidisciplinary Sciences
Johan Lundin Kleberg, Astrid E. Z. Hallman, Martyna A. Galazka, Deborah M. Riby, Sven Bolte, Charlotte Willfors, Christine Fawcett, Ann Nordgren
Summary: Typically developing individuals automatically adjust their pupil size to match others, while individuals with Williams syndrome lack this ability. This may explain the social challenges faced by individuals with Williams syndrome, despite their high social motivation.
SCIENTIFIC REPORTS
(2023)
Article
Behavioral Sciences
Michael W. Weiss, Megha Sharda, Miriam Lense, Krista L. Hyde, Sandra E. Trehub
Summary: Individuals with Autism Spectrum Disorder (ASD) and Williams syndrome (WS) show comparable memory advantages for vocal melodies compared to typically developing individuals, despite differences in overall memory performance. These results suggest enhanced processing of socially significant auditory signals in the context of music for individuals with ASD and WS.
Article
Psychology, Multidisciplinary
Maria Ashworth, Olympia Palikara, Elizabeth Burchell, Harry Purser, Dritan Nikolla, Jo Van Herwegen
Summary: The study compared cognitive assessment scores of children with Williams syndrome using online and face-to-face methods, finding higher BPVS scores for participants assessed online. The differences in task protocols were discussed in relation to these findings and their implications for neurodevelopmental research.
FRONTIERS IN PSYCHOLOGY
(2021)
Article
Psychology, Developmental
Johan Lundin Kleberg, Deborah Riby, Christine Fawcett, Hanna Bjorlin Avdic, Matilda A. Frick, Karin C. Brocki, Jens Hogstrom, Eva Serlachius, Ann Nordgren, Charlotte Willfors
Summary: Williams syndrome patients have difficulties in paying attention to others' eyes, which may impede social interaction skills.
JOURNAL OF AUTISM AND DEVELOPMENTAL DISORDERS
(2023)
Article
Medicine, General & Internal
Beth A. Kozel, Boaz Barak, Chong Ae Kim, Carolyn B. Mervis, Lucy R. Osborne, Melanie Porter, Barbara R. Pober
Summary: Williams syndrome is a rare genetic disorder caused by the microdeletion of a region of chromosome 7q11.23. It affects about 1:7,500 individuals, with cardinal features including cardiovascular disease, distinctive craniofacial appearance, intellectual disability, and hypersociability. Diagnosis at an earlier age due to technological advances has allowed for earlier intervention, but factors responsible for phenotypic variability remain unknown.
NATURE REVIEWS DISEASE PRIMERS
(2021)
Article
Psychology, Developmental
Lilja Kristin Jonsdottir, Janina Neufeld, Terje Falck-Ytter, Johan Lundin Kleberg
Summary: Studies have shown that individuals with Autism Spectrum Disorder (ASD) tend to avert their gaze from both eyes and mouths. This attentional avoidance is not specific to eyes and is not related to anxiety symptoms.
JOURNAL OF AUTISM AND DEVELOPMENTAL DISORDERS
(2023)
Article
Neurosciences
Charis Styliadis, Rachel Leung, Selin Ozcan, Eric A. Moulton, Elizabeth Pang, Margot J. Taylor, Christos Papadelis
Summary: The study found that adolescents with ASD showed overactivation in the left hemisphere of the cerebellum when processing happy facial expressions, and overactivation in the midline of the cerebellum when processing angry facial expressions. This indicates a prioritized hemispheric activity for happy faces and a later midline activity for angry faces in adolescents with ASD.
HUMAN BRAIN MAPPING
(2021)
Article
Mechanics
Huadong Zhang, Weichen Kong, Y. H. Liu, Yuh J. Chao
Summary: Williams' series expansion crack tip solution in linear elasticity is modified to include a uniform crack face pressure. Practical methods to calculate T-stress from near crack tip stresses are outlined. The analytical results are consistent with numerical results.
ENGINEERING FRACTURE MECHANICS
(2024)
Article
Psychology, Developmental
Bradley Powell, Jo Van Herwegen
Summary: This study explored individual differences and the development of sensory processing difficulties in children with Williams syndrome through two experiments. It found that sensory impairments were not stable across development, with impaired registration being a core phenotype in children with WS.
JOURNAL OF AUTISM AND DEVELOPMENTAL DISORDERS
(2022)
Article
Behavioral Sciences
Ayaka Ikeda, Yoko Hakuno, Kosuke Asada, Takahiro Ikeda, Takanori Yamagata, Masahiro Hirai
Summary: This study examined the developmental trajectories of emotion understanding in non-clinical (NC) children, children with autism spectrum disorder (ASD), and children with Williams syndrome (WS). The results showed that children with ASD followed a similar developmental path to NC children, while children with WS had different trajectories in certain aspects of emotion understanding, which were influenced by their Autism Spectrum Quotient scores.
Article
Behavioral Sciences
Rianne Haartsen, Luke Mason, Pilar Garces, Anna Gui, Tony Charman, Julian Tillmann, Mark H. Johnson, Jan K. Buitelaar, Eva Loth, Declan Murphy, Emily J. H. Jones
Summary: The study explores the neural processing of faces in individuals with autism and finds differences in the activation patterns between diagnostic groups. The findings suggest that early difficulties with configural face processing in autism may lead to compensatory processes and the involvement of non-typical neural systems in later development.
Article
Multidisciplinary Sciences
Johan Lundin Kleberg, Charlotte Willfors, Hanna Bjorlin Avdic, Deborah Riby, Martyna A. Galazka, Mona Guath, Ann Nordgren, Claes Strannegard
Summary: Williams syndrome (WS) is a rare genetic condition characterized by high social interest and approach motivation as well as intellectual disability and anxiety. Social feedback improves reward learning in individuals with WS but not in typically developing controls or individuals with other intellectual disabilities. Computational modeling suggests that this effect is mediated by a shift towards higher weight given to rewards and increased choice consistency.
SCIENTIFIC REPORTS
(2023)
Article
Education, Special
Rebecca M. Kirchner, Katherine M. Walton
Summary: Research suggests that children with DS or WS exhibit higher levels of ASD symptoms compared to the general population, particularly in the area of unusual behaviors. Possible explanations for these elevations include issues with measurement, etiological overlap, or similar behavioral phenotypes. Further research is needed to enhance our understanding of the overlap of ASD symptoms in these populations.
AJIDD-AMERICAN JOURNAL ON INTELLECTUAL AND DEVELOPMENTAL DISABILITIES
(2021)
Article
Psychiatry
Andrea C. Samson, Nayla Sokhn, Jo Van Herwegen, Daniel Dukes
Summary: This study explores emotion regulation strategy use in individuals with different neurodevelopmental disorders, revealing specific group differences in the use of strategies and identifying certain strategies linked to elevated anxiety levels while focusing on the positive is associated with lower anxiety levels in all groups.
FRONTIERS IN PSYCHIATRY
(2022)
Article
Ophthalmology
Nitzan Guy, Yoni Pertzov
Summary: This study explores the reliance of humans on the visual and oculomotor systems during face-to-face interactions. It investigates individual differences in gaze behavior during screen-based and live interviews, and how it is related to social anxiety, autism, and neuroticism. The study distinguishes between the tendency to look at the face and the eyes and finds high internal consistencies within both interview scenarios. The findings suggest that individual variations in gaze behavior are robust across different interview scenarios, and measuring the tendency to look at faces separately from the eyes is useful.
Article
Medicine, Research & Experimental
Victoria Grahame, Linda Dixon, Sue Fletcher-Watson, Deborah Garland, Magdalena Glod, Jane Goodwin, Zoe Grayson, Saoirse Heron, Emma Honey, Rebecca Iversen, Adetayo S. Kasim, Ashleigh Kernohan, Ehsan Kharatikoopaei, Ann Le Couteur, Leila Mackie, Ayesha Mathias, Helen Probert, Deborah Riby, Priyanka Rob, Leanne Rogan, Sarah Thompson, Luke Vale, Eamonn Walls, Elspeth Imogen Webb, Christopher Weetman, Faye Wolstenhulme, Ruth Wood, Jacqui Rodgers
Summary: The study is a clinical and cost-effectiveness, multi-site randomised controlled trial comparing the Managing Repetitive Behaviours (MRB) parent group intervention with a psychoeducation parent group Learning About Autism (LAA) for families of young children with autism. The primary outcome using generalised estimating equation will compare the proportion of children with improved RRB between the two groups.
Article
Behavioral Sciences
Mikle South, Mary Hanley, Karys Normansell-Mossa, Nicholas C. C. Russell, Thomas Cawthorne, Deborah M. Riby
Summary: Autism and Williams Syndrome share similarities in social and anxiety traits, but also have key differences. The intolerance of uncertainty affects the relationship between social profiles and anxiety in children diagnosed with either condition.
Article
Psychology, Developmental
V Sideropoulos, D. Dukes, M. Hanley, O. Palikara, S. Rhodes, D. M. Riby, A. C. Samson, J. Van Herwegen
Summary: This study found that during the first UK national lockdown, parents reported increased anxiety levels for themselves and their children with Special Education Needs and Disabilities (SEND), with the anxiety levels in the SEND group predicted by awareness about COVID-19. Worries differed between the groups, indicating that COVID-19 impacts the wellbeing of those with SEND differently to that of their typically developing siblings.
JOURNAL OF AUTISM AND DEVELOPMENTAL DISORDERS
(2022)
Article
Genetics & Heredity
Charlotte Willfors, Deborah M. Riby, Marcus van der Poll, Katja Ekholm, Hanna Avdic Bjorlin, Johan Lundin Kleberg, Ann Nordgren
Summary: Individuals with Williams syndrome have an elevated risk for anxiety disorders throughout life. This study aimed to investigate the effects of intelligence, inhibition, sustained attention, and working memory on anxiety in WS using direct measures. The results showed that lower IQ and older age were associated with elevated anxiety, while there was no significant association between anxiety and core elements of cognitive executive functions.
ORPHANET JOURNAL OF RARE DISEASES
(2021)
Article
Psychology, Developmental
Johan Lundin Kleberg, Deborah Riby, Christine Fawcett, Hanna Bjorlin Avdic, Matilda A. Frick, Karin C. Brocki, Jens Hogstrom, Eva Serlachius, Ann Nordgren, Charlotte Willfors
Summary: Williams syndrome patients have difficulties in paying attention to others' eyes, which may impede social interaction skills.
JOURNAL OF AUTISM AND DEVELOPMENTAL DISORDERS
(2023)
Article
Education, Special
Ellen Ridley, Bronia Arnott, Deborah M. Riby, D. Michael Burt, Mary Hanley, Susan R. Leekam
Summary: Past research has shown that individuals with Williams syndrome (WS) have heightened and prolonged eye contact. This study used parent report measures to examine both the presence and quality of eye contact. Unusual eye contact features were found in approximately half of the WS samples, while other features like brief glances were common in WS and all other neurodevelopmental conditions but not in typically developing individuals. Future research should focus on both the qualitative and quantitative aspects of eye contact in neurodevelopmental conditions.
AJIDD-AMERICAN JOURNAL ON INTELLECTUAL AND DEVELOPMENTAL DISABILITIES
(2022)
Article
Psychology, Developmental
Amanda E. Gillooly, Deborah M. Riby, Kevin Durkin, Sinead M. Rhodes
Summary: Children with Williams syndrome (WS) often face challenges in making friends, possibly due to difficulties in play and self-regulation. However, a small number of children are able to form strong friendships.
JOURNAL OF AUTISM AND DEVELOPMENTAL DISORDERS
(2022)
Article
Education, Special
Catherine E. Stanford, Richard P. Hastings, Deborah M. Riby, Heather J. Archer, Sarah E. Page, Katie Cebula
Summary: This study compared mothers who were parenting a child with ASD and other neurodevelopmental disorders to mothers who were parenting a child with ASD and typically developing children. The results showed no significant differences in mental health between the two groups, but the mothers in the ASD-NDD group reported greater positive gain.
INTERNATIONAL JOURNAL OF DEVELOPMENTAL DISABILITIES
(2022)
Article
Psychology, Multidisciplinary
Joanna M. H. Greer, Deborah M. Riby, Mhairi E. G. McMullon, Colin Hamilton, Leigh M. Riby
Summary: This study investigated neural mechanisms during resting states in adults with Williams syndrome (WS) using EEG methodology. The results showed a trend of lower alpha power in the WS group compared to controls, but no significant difference in beta power. Additionally, an unusual low variability in the EEG signature of the WS group was found, contradicting the typically observed behavioral heterogeneity.
Article
Psychology, Developmental
Amanda E. Gillooly, Deborah M. Riby, Kevin Durkin, Sinead M. Rhodes
Summary: The study found that children with Williams syndrome (WS) experience difficulties in peer relations, including sustaining friendships and social exclusion. Greater social functioning difficulties were associated with more significant peer relation problems.
JOURNAL OF AUTISM AND DEVELOPMENTAL DISORDERS
(2021)
Article
Education, Special
Emily McDougal, Deborah M. Riby, Mary Hanley
RESEARCH IN DEVELOPMENTAL DISABILITIES
(2020)
Article
Education, Special
Emily McDougal, Deborah M. Riby, Mary Hanley
RESEARCH IN AUTISM SPECTRUM DISORDERS
(2020)
Article
Education, Special
Pamela Heaton, Ellen Ridley, Sonya Makhmood, Deborah M. Riby
RESEARCH IN DEVELOPMENTAL DISABILITIES
(2020)
Article
Education, Special
M. Glod, D. M. Riby, J. Rodgers
JOURNAL OF INTELLECTUAL DISABILITY RESEARCH
(2020)
Article
Education, Special
Ellen Ridley, Deborah M. Riby, Susan R. Leekam
RESEARCH IN DEVELOPMENTAL DISABILITIES
(2020)
