Behavioural features of Italian infants and young adults with Williams-Beuren syndrome.
Inattention and anxiety are everyday facts of life for people with Williams-Beuren syndrome, while self-absorbed behaviors fade with school structure.
01Research in Context
What this study did
Doctors in Italy watched 24 people with Williams-Beuren syndrome. Ages ranged from babies to adults.
They used parent forms and clinic notes to list each person's behavior problems.
What they found
Almost every person had trouble paying attention. Most also showed strong worry and loud outbursts.
Younger kids often stared into space or rocked. These self-absorbed actions faded once school started.
How this fits with other research
Kocher et al. (2015) asked parents and teachers the same questions and got the same answers. Both studies say attention and anxiety are core traits in Williams syndrome.
Van Hanegem et al. (2014) dug deeper into sensory issues. They found itchy tags, loud vacuums, and hand-flapping are also common. Spanoudis et al. (2011) only skimmed these signs, so the 2014 paper extends the picture.
Ahlborn et al. (2008) tested sound reactivity in a lab. Nine out of ten kids with Williams syndrome startled at soft tones. The Italian study saw the same jumpy reactions in daily life. Lab or living room, the ears stay on high alert.
Why it matters
If you assess a child with Williams syndrome, plan for inattention and anxiety first. Use short work periods and clear signals. Add sound dampening or headphones if fire alarms or hand dryers trigger meltdowns. When school adds structure, self-stimming drops, so push for classroom routines and visual schedules.
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02At a glance
03Original abstract
BACKGROUND: The increased interest in social interaction in Williams-Beuren syndrome (WBS) is evident from infancy onwards, together not only with increased empathy, positive interpersonal bias, but also with social disinhibition. Previous studies have described behavioural and emotional problems as being widely represented in WBS. There is limited scope for comparisons between literature data because of the variety of instruments used to assess behaviour. METHOD: Forty-one children and young adults with WBS were enrolled and underwent general cognitive assessment. In order to compare our data with the literature, we used standardised questionnaires used in previous studies (Developmental Behaviour Checklist: DBC-P). General cognitive abilities, gender and age were included in the analysis. RESULTS: Behavioural problems were more relevant than expected according to intellectual impairment. Some features were present at any age: inattention, anxiety, disruptive behaviours. Antisocial conduct was almost absent; perseverative conduct, a poor sense of danger and, more generally, self-absorbed behaviours tended to diminish along with age and to be linked to more pronounced cognitive impairment. CONCLUSION: As previously described for other countries, behaviour disturbances occur frequently in the Italian WBS population. Our data could support the existence of some 'intrinsic' behavioural characteristics in WBS such as inattention and anxiety, which are detectable and important at any age; both learning and social exposure to a structured context such as school could help diminish self-absorbed behaviour.
Journal of intellectual disability research : JIDR, 2011 · doi:10.1111/j.1365-2788.2010.01376.x