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Pitts, C. Holley; Mervis, Carolyn B. – American Journal on Intellectual and Developmental Disabilities, 2016
We describe the performance of 292 4- to 17-year-olds with Williams syndrome (WS) on the Kaufman Brief Intelligence Test-2 (KBIT-2; Kaufman & Kaufman, 2004). Mean IQ Composite, Verbal standard score (SS), and Nonverbal SS were in the borderline range relative to the general population, with variability similar to the general population.…
Descriptors: Intelligence Tests, Genetic Disorders, Intelligence Quotient, Children
Chevalère, Johann; Postal, Virginie; Jauregui, Joseba; Copet, Pierre; Laurier, Virginie; Thuilleaux, Denise – American Journal on Intellectual and Developmental Disabilities, 2015
The aim of this study was to support the growing evidence suggesting that Prader-Willi Syndrome (PWS) might present with an impairment of executive functions (EFs) and to investigate whether this impairment is specific to patients with PWS or due to their intellectual disability (ID). Six tasks were administered to assess EFs (inhibition,…
Descriptors: Genetic Disorders, Disabilities, Executive Function, Neurological Impairments
Turkstra, Lyn S.; Abbeduto, Leonard; Meulenbroek, Peter – American Journal on Intellectual and Developmental Disabilities, 2014
This study aimed to characterize social cognition, executive functions (EFs), and everyday social functioning in adolescent girls with fragile X syndrome, and identify relationships among these variables. Participants were 20 girls with FXS and 20 age-matched typically developing peers. Results showed significant between-groups differences in…
Descriptors: Executive Function, Social Cognition, Interpersonal Competence, Females
Hogan-Brown, Abigail L.; Losh, Molly; Martin, Gary E.; Mueffelmann, Deborah J. – American Journal on Intellectual and Developmental Disabilities, 2013
Whereas pragmatic language difficulties are characteristic of both autism and Fragile X syndrome, it is unclear whether such deficits are qualitatively similar or whether certain skills are differentially affected. This study compared narrative competence in boys with autism, Fragile X syndrome, Down syndrome, and typical development. Results…
Descriptors: Males, Story Telling, Autism, Pervasive Developmental Disorders
Fisch, Gene S.; Carpenter, Nancy; Howard-Peebles, Patricia N.; Holden, Jeanette J. A.; Tarleton, Jack; Simensen, Richard; Battaglia, Agatino – American Journal on Intellectual and Developmental Disabilities, 2012
Few studies exist of developmental trajectories in children with intellectual disability, and none for those with subtelomeric deletions. We compared developmental trajectories of children with Wolf-Hirschhorn syndrome to other genetic disorders. We recruited 106 children diagnosed with fragile X, Williams-Beuren syndrome, or Wolf-Hirschhorn…
Descriptors: Mental Retardation, Genetic Disorders, Congenital Impairments, Child Development
Sterling, Audra M.; Warren, Steven F.; Brady, Nancy; Fleming, Kandace – American Journal on Intellectual and Developmental Disabilities, 2013
This study investigated the influence of maternal and child variables on the maternal responsivity of 55 mothers with young children with fragile X syndrome. Data included video observations of mother-child interactions in four different contexts, standardized assessments with the children, and standardized questionnaires for the mothers. The…
Descriptors: Genetic Disorders, Mothers, Young Children, Observation
Mervis, Carolyn B.; Kistler, Doris J.; John, Angela E.; Morris, Colleen A. – American Journal on Intellectual and Developmental Disabilities, 2012
Multilevel modeling was used to address the longitudinal stability of standard scores (SSs) measuring intellectual ability for children with Williams syndrome (WS). Participants were 40 children with genetically confirmed WS who completed the Kaufman Brief Intelligence Test--Second Edition (KBIT-2; A. S. Kaufman & N. L. Kaufman, 2004) 4-7…
Descriptors: Congenital Impairments, Genetic Disorders, Mental Retardation, Intelligence Quotient
Cornish, Kim; Cole, Victoria; Longhi, Elena; Karmiloff-Smith, Annette; Scerif, Gaia – American Journal on Intellectual and Developmental Disabilities, 2012
Basic attentional processes and their impact on developmental trajectories in fragile X syndrome were assessed in a 3-year prospective study. Although fragile X syndrome is a monogenic X-linked disorder, there is striking variability in outcomes even in young boys with the condition. Attention is a key factor constraining interactions with the…
Descriptors: Genetic Disorders, Mental Retardation, Congenital Impairments, Attention
McDuffie, Andrea; Kover, Sara; Abbeduto, Leonard; Lewis, Pamela; Brown, Ted – American Journal on Intellectual and Developmental Disabilities, 2012
The authors examined receptive and expressive language profiles for a group of verbal male children and adolescents who had fragile X syndrome along with varying degrees of autism symptoms. A categorical approach for assigning autism diagnostic classification, based on the combined use of the Autism Diagnostic Interview--Revised and the Autism…
Descriptors: Receptive Language, Expressive Language, Language Skills, Profiles
Stinton, Chris; Elison, Sarah; Howlin, Patricia – American Journal on Intellectual and Developmental Disabilities, 2010
Although many researchers have investigated emotional and behavioral difficulties in individuals with Williams syndrome, few have used standardized diagnostic assessments. We examined mental health problems in 92 adults with Williams syndrome using the Psychiatric Assessment Schedule for Adults with Developmental Disabilities--PAS-ADD (Moss,…
Descriptors: Developmental Disabilities, Mental Health, Depression (Psychology), Mental Disorders
McDuffie, Andrea; Abbeduto, Leonard; Lewis, Pamela; Kover, Sara; Kim, Jee-Seon; Weber, Ann; Brown, W. Ted – American Journal on Intellectual and Developmental Disabilities, 2010
The Autism Diagnostic Interview-Revised (ADI-R) was used to examine diagnostic profiles and age-related changes in autism symptoms for a group of verbal children and adolescents who had fragile X syndrome, with and without autism. After controlling for nonverbal IQ, we found statistically significant between-group differences for lifetime and…
Descriptors: Autism, Interests, Intelligence Quotient, Interaction