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Visual and visuospatial development in young children with Williams syndrome
J Atkinson1, S Anker, O Braddick
1Department of Psychology, University College London, UK. j.atkinson@ucl.ac.uk
Insights
Children with Williams syndrome (WS) have frequent vision problems, but these do not explain their visuospatial difficulties. This suggests a specific deficit in how their brains process spatial information.
Area of Science:
- Neuroscience
- Developmental Psychology
- Ophthalmology
Background:
- Williams syndrome (WS) is a genetic disorder associated with cognitive and behavioral differences.
- Children with WS often exhibit visuospatial deficits and may have co-occurring sensory vision problems.
Purpose of the Study:
- To investigate the relationship between sensory visual problems and visuospatial difficulties in young children with WS.
- To determine if sensory vision deficits contribute to the severity of visuospatial challenges in WS.
Main Methods:
- A cohort of 108 children with WS (mean age 7 years 3 months) had their families complete a vision problems questionnaire.
- Detailed visual, spatial, motor, visuocognitive, and linguistic assessments were performed on 73 children with WS.
- Statistical analysis examined correlations between sensory vision deficits and visuospatial test performance.
Main Results:
- Children with WS demonstrated a higher prevalence of common pediatric sensory vision problems (e.g., strabismus, amblyopia) compared to typically developing children.
- No significant correlation was found between the presence of sensory visual deficits and the severity of visuospatial problems in children with WS.
- Visuospatial and language abilities were dissociated, supporting a neurobiological model of distinct visual processing streams.
Conclusions:
- The visuospatial difficulties in Williams syndrome are not solely attributable to sensory visual impairments.
- Findings support a generalized deficit in dorsal stream visual processing in young children with WS.
- This dissociation highlights specific neurodevelopmental differences in visual information processing in WS.
Abstract:
This study investigated the relation between sensory visual problems and the severity of visuospatial difficulties in a large group of young children with Williams' syndrome (WS). A questionnaire describing visual and associated problems was completed by the families of 108 children with WS and detailed follow-up assessments were conducted, including visual, spatial, motor, visuocognitive, and linguistic tests of 73 of these children (mean age 7 years 3 months; 40 males, 73 females). Children with WS showed a much higher incidence of common paediatric sensory vision problems (strabismus, visual acuity loss, amblyopia, reduced stereopsis) than normally developing children. It was found that delays with respect to age normative values increased with age on all tests. No significant correlation was found between the presence of a visual deficit and the severity of the visuospatial problems, suggesting that the difficulties children with WS have in understanding spatial arrangements are not simply a result of their earlier sensory visual problems. Results confirm the dissociation between visuospatial and language abilities in children with WS, and support the neurobiological model of a split between ventral and dorsal stream processing of visual information with a generalized deficit in dorsal stream processing in young children with WS.