Cognitive and behavioral profile of Williams Syndrome toddlers

Abstract

Purpose To verify indicators of cognitive development, receptive language skills and adaptive behavioral patterns in toddlers with Williams syndrome (WS). Methods The sample comprised 8 children of both sex, aged between 48 and 72 months with WS. Instruments of data collection were Denver Developmental Screening Test II; Peabody Picture Vocabulary Test; Vineland Adaptive Behavior Scale; Child Behavior Checklist for Ages 1½-5and 6 to 18; Columbia Mental Maturity Scale (CMMS), and Behavior Problems Inventory-01. Results The major developmental impairments were associated with fine motor skills and personal care abilities. Deficits in receptive language and communication skills were reported according to the PPVT and Denver II, respectively. The caregivers reported behavioral and emotional problems associated to anxiety and depression, and attention problems scales of CBCL. Conclusion The toddlers demonstrated deficits in adaptive functioning and behavioral, motor and cognitive difficulties such as inattention and hyperactivity, stereotypies and aggressive behavior.

General Measurement Tools for Assessing Mental Health Problems Among Children and Adolescents with an Intellectual Disability: A Systematic Review

“…A total of 49 papers reporting on 10 instruments for assessing MH problems among children and adolescents with ID were identified and included (Aman et al, 1996 ; Baraldi et al, 2013 ; Borthwick-Duffy et al, 1997 ; Bostrom et al, 2016 ; Braga et al, 2018 ; Brereton et al, 2006 ; Brown et al, 2002 ; Chadwick et al, 2000 ; Clarke et al, 2003 ; Coe et al, 1999 ; Dekker et al, 2002a , 2002b , 2002c ; Dieleman et al, 2018 ; Douma et al, 2006 ; Einfeld & Tonge, 1995 ; El-Keshky & Emam, 2015 ; Embregts et al, 2010 ; Emerson, 2005 ; Esbensen et al, 2018 ; Freund & Reiss, 1991 ; Hassiotis & Turk, 2012 ; Hastings et al, 2001 ; Haynes et al, 2013 ; Jacola et al, 2014 ; Kaptein et al, 2008 ; Koskentausta & Almqvist, 2004 ; Koskentausta et al, 2004 ; Marshburn & Aman, 1992 ; Masi et al, 2002 ; Matson et al, 1984 ; Mircea et al, 2010 ; Murray et al, 2020 ; Norris & Lecavalier, 2011 ; Oliver et al, 2003 ; Oubrahim & Combalbert, 2019 ; Reiss & Valenti-Hein, 1994 ; Rice et al, 2018 ; Rojahn & Helsel, 1991 ; Rojahn et al, 2010 ; Sansone et al, 2012 ; Taffe et al, 2007 ; Tasse & Lecavalier, 2000 ; Tasse et al, 1996 ; Tonge et al, 1996 ; van Lieshout et al, 1998 ; Wallander et al, 2006 ; Wolf, 1981 ; Wright, 2010 ) (see Tables 2 and 3 ). Of these instruments, seven were aimed for the intellectual and developmental disability (IDD) population (i.e., ID instruments), while three instruments were ...…”

“…52% boys. Age range 8–20 years Mild to profound ID FSIQ: NR 67 Cross-sectional Parent Factor structure (EFA) CBCL Braga et al ( 2018 ) Brazil Williams syndrome 38% boys. Age range 4–6 years NR 8 Cross-sectional Parent/caregiver Convergent validity (BPI-01) CBCL and TRF Dekker et al ( 2002a ) a Netherlands Special education.…”

“…A total of 49 papers reporting on 10 instruments for assessing MH problems among children and adolescents with ID were identified and included (Aman et al, 1996;Baraldi et al, 2013;Borthwick-Duffy et al, 1997;Bostrom et al, 2016;Braga et al, 2018;Brereton et al, 2006;Brown et al, 2002;Chadwick et al, 2000;Clarke et al, 2003;Coe et al, 1999;Dekker et al, 2002aDekker et al, , 2002bDekker et al, , 2002cDieleman et al, 2018;Douma et al, 2006;El-Keshky & Emam, 2015;Embregts et al, 2010;Emerson, 2005;Esbensen et al, 2018;Freund & Reiss, 1991;Hassiotis & Turk, 2012;Hastings et al, 2001;Haynes et al, 2013;Jacola et al, 2014;Kaptein et al, 2008;Marshburn & Aman, 1992;Masi et al, 2002;Matson et al, 1984;Mircea et al, 2010;Murray et al, 2020;Norris & Lecavalier, 2011;Oliver et al, 2003;Reiss & Valenti-Hein, 1994;Rice et al, 2018;Rojahn & Helsel, 1991;Sansone et al, 2012;Taffe et al, 2007;Tasse &...…”

Cognitive profile of young children with Williams syndrome

“…1996; Braga et al . 2018). Further, where studies explore multiple cognitive domains, they often report small sample sizes, and different tests are often used depending on the child's or adult's age, which again means test scores should not be directly compared.…”

“…1984; Braga et al . 2018). Assessment of individual variability is important, as it informs researchers, clinicians and educators on the possible demographic and environmental contributions to early development and informs the development of individualised management programmes for those with WS.…”