ArticleMolecular genetics & genomic medicine2019
Cognition in adults with Williams syndrome-A 20-year follow-up study.
Article in Molecular genetics & genomic medicine, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers, 1 of them a synthesis that pooled it.
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Who cites it
9 citing papers in PubMed, 1 synthesis or guideline pooled it, 20 citations in OpenAlex.
- Oxytocin and Oxytocin Receptor Gene Regulation in Williams Syndrome: A Systematic Review.The Yale journal of biology and medicine · 2021Pooled it
- Eating Disorder Screening in Adults With Williams Syndrome: A Preliminary Report.The International journal of eating disorders · 2026Article
- Observational study to preliminarily characterize the audiological profile of chinese children with williams syndrome.Journal of otology · 2025Article
- [Characteristics of early cognitive development in children with Williams syndrome: a prospective cohort study].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2024Article
- Prenatal alcohol exposure and cognition at midlife: Evidence of fluid cognition deficits in two cohorts.Alcohol, clinical & experimental research · 2023Article
- Williams syndrome.Nature reviews. Disease primers · 2021Review
- A cross-comparison of cognitive ability across 8 genomic disorders.Current opinion in genetics & development · 2021Review
- Cognition in adults with Williams syndrome-A 20-year follow-up study.Molecular genetics & genomic medicine · 2019Article
- [Early motor development in children with Williams syndrome].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatricsArticle
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Authors and funding
5 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundWilliams syndrome (WBS) is a genetic multisystem disorder. The main symptom is borderline (intelligence quotient, IQ 70-79) or abnormally low intelligence (IQ < 70). According to earlier studies young individuals with WBS demonstrate generally a slightly higher verbal IQ (VIQ) compared to performance/nonverbal IQ (PIQ). WBS was recognized as a distinct entity already about 60 years ago, but still cognition in adults with WBS is poorly known.
methodsWe followed 25 adults (age at baseline 19-68, median 38) with genetically confirmed WBS for about 20 years. The study subjects underwent medical and neuropsychological assessments at the baseline and at the end of follow-up.
resultsThe mean VIQ remained quite stable from early adulthood up to 40 years of age after which it declined. The mean PIQ kept on improving from early adulthood until 50 years of age after which it gradually declined. At the end of the study, all study subjects had at least two longstanding health problems out of which hypertension, psychiatric disorder, and scoliosis or kyphosis occurred most frequently. At end of the study, two patients suffered from vascular dementia. Seven patients died during the follow-up.
conclusionsIn adults with WBS, the course of cognition is uneven across the cognitive profile. Their verbal functions both develop and deteriorate earlier than performance/nonverbal functions. Frequent somatic co-morbidities may increase risk to shortened life span.
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