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Cognition in adults with Williams syndrome—A 20‐year follow‐up study

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Zeitschriftentitel: Molecular Genetics & Genomic Medicine
Personen und Körperschaften: Sauna‐aho, Oili, Bjelogrlic‐Laakso, Nina, Sirén, Auli, Kangasmäki, Virpi, Arvio, Maria
In: Molecular Genetics & Genomic Medicine, 7, 2019, 6
Format: E-Article
Sprache: Englisch
veröffentlicht:
Wiley
Schlagwörter:
Genetics (clinical)
Genetics
Molecular Biology
author_facet Sauna‐aho, Oili
Bjelogrlic‐Laakso, Nina
Sirén, Auli
Kangasmäki, Virpi
Arvio, Maria
Sauna‐aho, Oili
Bjelogrlic‐Laakso, Nina
Sirén, Auli
Kangasmäki, Virpi
Arvio, Maria
author Sauna‐aho, Oili
Bjelogrlic‐Laakso, Nina
Sirén, Auli
Kangasmäki, Virpi
Arvio, Maria
spellingShingle Sauna‐aho, Oili
Bjelogrlic‐Laakso, Nina
Sirén, Auli
Kangasmäki, Virpi
Arvio, Maria
Molecular Genetics & Genomic Medicine
Cognition in adults with Williams syndrome—A 20‐year follow‐up study
Genetics (clinical)
Genetics
Molecular Biology
author_sort sauna‐aho, oili
spelling Sauna‐aho, Oili Bjelogrlic‐Laakso, Nina Sirén, Auli Kangasmäki, Virpi Arvio, Maria 2324-9269 2324-9269 Wiley Genetics (clinical) Genetics Molecular Biology http://dx.doi.org/10.1002/mgg3.695 <jats:title>Abstract</jats:title><jats:sec><jats:title>Background</jats:title><jats:p>Williams syndrome (WBS) is a genetic multisystem disorder. The main symptom is borderline (intelligence quotient, IQ 70–79) or abnormally low intelligence (IQ &lt; 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.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>We 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.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>The 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.</jats:p></jats:sec><jats:sec><jats:title>Conclusions</jats:title><jats:p>In 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.</jats:p></jats:sec> Cognition in adults with Williams syndrome—A 20‐year follow‐up study Molecular Genetics & Genomic Medicine
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title Cognition in adults with Williams syndrome—A 20‐year follow‐up study
title_unstemmed Cognition in adults with Williams syndrome—A 20‐year follow‐up study
title_full Cognition in adults with Williams syndrome—A 20‐year follow‐up study
title_fullStr Cognition in adults with Williams syndrome—A 20‐year follow‐up study
title_full_unstemmed Cognition in adults with Williams syndrome—A 20‐year follow‐up study
title_short Cognition in adults with Williams syndrome—A 20‐year follow‐up study
title_sort cognition in adults with williams syndrome—a 20‐year follow‐up study
topic Genetics (clinical)
Genetics
Molecular Biology
url http://dx.doi.org/10.1002/mgg3.695
publishDate 2019
physical
description <jats:title>Abstract</jats:title><jats:sec><jats:title>Background</jats:title><jats:p>Williams syndrome (WBS) is a genetic multisystem disorder. The main symptom is borderline (intelligence quotient, IQ 70–79) or abnormally low intelligence (IQ &lt; 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.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>We 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.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>The 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.</jats:p></jats:sec><jats:sec><jats:title>Conclusions</jats:title><jats:p>In 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.</jats:p></jats:sec>
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author Sauna‐aho, Oili, Bjelogrlic‐Laakso, Nina, Sirén, Auli, Kangasmäki, Virpi, Arvio, Maria
author_facet Sauna‐aho, Oili, Bjelogrlic‐Laakso, Nina, Sirén, Auli, Kangasmäki, Virpi, Arvio, Maria, Sauna‐aho, Oili, Bjelogrlic‐Laakso, Nina, Sirén, Auli, Kangasmäki, Virpi, Arvio, Maria
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description <jats:title>Abstract</jats:title><jats:sec><jats:title>Background</jats:title><jats:p>Williams syndrome (WBS) is a genetic multisystem disorder. The main symptom is borderline (intelligence quotient, IQ 70–79) or abnormally low intelligence (IQ &lt; 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.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>We 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.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>The 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.</jats:p></jats:sec><jats:sec><jats:title>Conclusions</jats:title><jats:p>In 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.</jats:p></jats:sec>
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spelling Sauna‐aho, Oili Bjelogrlic‐Laakso, Nina Sirén, Auli Kangasmäki, Virpi Arvio, Maria 2324-9269 2324-9269 Wiley Genetics (clinical) Genetics Molecular Biology http://dx.doi.org/10.1002/mgg3.695 <jats:title>Abstract</jats:title><jats:sec><jats:title>Background</jats:title><jats:p>Williams syndrome (WBS) is a genetic multisystem disorder. The main symptom is borderline (intelligence quotient, IQ 70–79) or abnormally low intelligence (IQ &lt; 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.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>We 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.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>The 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.</jats:p></jats:sec><jats:sec><jats:title>Conclusions</jats:title><jats:p>In 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.</jats:p></jats:sec> Cognition in adults with Williams syndrome—A 20‐year follow‐up study Molecular Genetics & Genomic Medicine
spellingShingle Sauna‐aho, Oili, Bjelogrlic‐Laakso, Nina, Sirén, Auli, Kangasmäki, Virpi, Arvio, Maria, Molecular Genetics & Genomic Medicine, Cognition in adults with Williams syndrome—A 20‐year follow‐up study, Genetics (clinical), Genetics, Molecular Biology
title Cognition in adults with Williams syndrome—A 20‐year follow‐up study
title_full Cognition in adults with Williams syndrome—A 20‐year follow‐up study
title_fullStr Cognition in adults with Williams syndrome—A 20‐year follow‐up study
title_full_unstemmed Cognition in adults with Williams syndrome—A 20‐year follow‐up study
title_short Cognition in adults with Williams syndrome—A 20‐year follow‐up study
title_sort cognition in adults with williams syndrome—a 20‐year follow‐up study
title_unstemmed Cognition in adults with Williams syndrome—A 20‐year follow‐up study
topic Genetics (clinical), Genetics, Molecular Biology
url http://dx.doi.org/10.1002/mgg3.695