Record 1Corpus callosum morphology of Williams syndrome: relation to genetics and behavior. Schmitt JE; Eliez S; Warsofsky IS; Bellugi U; Reiss AL Department of Psychiatry and Behavioral Sciences, Stanford University School of Medicine, CA 94305-5719, USA. Developmental medicine and child neurology (England) Mar 2001, 43 (3) p155-9, ISSN 0012-1622 Languages: ENGLISH Document type: Journal Article
As the largest interhemispheric commissure in the brain, the corpus callosum is of particular interest in disorders that may preferentially affect white matter development such as Williams syndrome (WS). Individuals with WS possess a remarkable array of neurobehavioral peaks and valleys, including deficits in visuospatial ability, mathematics, and attention, but with relative preservation of language and affect. Our study measured the corpus callosum and its primary subdivisions using high-resolution MRI in 20 individuals with WS (13 females and seven males; mean age 28.5, SD 8.3 years; range 19 to 44 years) and 20 age- and sex-matched control participants (mean age 28.5, SD 8.2 years; range 19 to 48 years). Total midsagittal corpus callosum area was reduced (F=4.5, p=0.04, df=36) in the WS population. The area of the splenium (F=12.4, p=0.001, df=36) and isthmus (F=9.4, p=0.004, df=36) were disproportionately reduced in WS beyond the absolute reduction of the entire corpus callosum. These reductions are in concordance with other neuroanatomical findings of decreased parietooccipital volumes as well as the observed visuospatial problems associated with WS.
Record 2[In Process Citation] Williams-syndroom: nieuwe inzichten in genetische etiologie, pathogenese en kliniek. van Hagen JM; Govaerts LC; de Coo IF; Gille JJ; Nieuwint AW; Madan K VU Medisch Centrum, afd. Klinische Genetica en Antropogenetica, Postbus 7057, 1007 MB Amsterdam. [email protected] Nederlands tijdschrift voor geneeskunde (Netherlands) Mar 3 2001, 145 (9) p396-400, ISSN 0028-2162 Languages: DUTCH Document type: Journal Article
Williams syndrome (WS) is a developmental disorder characterized by distinct facial features, congenital heart disease, mental retardation and a gregarious personality. The majority of people with this disorder have a submicroscopic deletion of genes in chromosome band 7q11.23. This deletion can be detected using fluorescence in situ hybridization (FISH). Although the condition is usually sporadic a few familial cases with autosomal dominant inheritance have been described. A clinical scoring system has been developed by Selicorni with which a diagnosis of 'Williams syndrome' can be made; in all patients in whom the diagnosis was made in this way FISH results were positive.
Record 3Drawings by individuals with Williams syndrome: are people different from shapes? Dykens EM; Rosner BA; Ly TM Neuropsychiatric Institute and Hospital, Division of Child and Adolescent Psychiatry, University of California, Los Angeles 90024-1759, USA. American journal of mental retardation (United States) Jan 2001, 106 (1) p94-107, ISSN 0895-8017 Languages: ENGLISH Document type: Journal Article
Because it is unclear whether people with Williams syndrome produce drawings that are delayed or deviant, we examined these two possibilities in Draw a Person and figure copying tasks (VMI) in 28 persons with Williams syndrome, 28 with mixed etiologies, and 28 with Down syndrome. All human figures could be classified into discrete stages of drawing development, and in all groups, drawing tasks were significantly correlated with MA. Human figures from participants with Williams syndrome were no more deviant than their counterparts, nor did they show "local-global" differences. Draw a Person scores exceeded VMI scores in the Williams syndrome group, whereas the Down syndrome group showed relative strengths on both drawing tasks, and the mixed group had no profile. Developmental and phenotypic implications of findings are discussed.
Record 4Longitudinal course of behavioral and emotional problems in Williams syndrome. Einfeld SL; Tonge BJ; Rees VW University of New South Wales, Sydney, Australia. [email protected] American journal of mental retardation (United States) Jan 2001, 106 (1) p73-81, ISSN 0895-8017 Document type: Journal Article
A follow-up study of behavior and emotional problems in a cohort of young people with Williams syndrome 5 years after first assessment is described. Using a between-/within-subjects factorial layout, we compared scores on the Developmental Behaviour Checklist between young people with Williams syndrome and a large epidemiological control sample of young people with mental retardation due to other causes from Time 1 (1990/1991) to Time 2 (1995/1996). Results showed substantial persistence of the overall level of behavior and emotional problems. However, there were changes in certain types of behavior. Participants with Williams syndrome had significantly higher overall behavioral and emotional problems, communication disturbance, and anxiety over the 5-year period. Further, 10 or 13 checklist items maintained significantly higher levels among the Williams syndrome sample.
Record 5Treating food refusal in a child with Williams syndrome using the parent as therapist in the home setting. O'Reilly M; Lancioni G 1 National University of Ireland, Dublin, Ireland; 2 University of Leiden, Leiden, the Netherlands. Journal of intellectual disability research (England) Feb 2001, 45 (Pt 1) p41-6, ISSN 0964-2633 Languages: ENGLISH Document type: Journal Article
The present authors examined the effectiveness of a behavioural intervention which included escape extinction and differential reinforcement of each bite eaten to treat non-organic food refusal in a child with Williams syndrome. The intervention was implemented by the child's mother in the home during normal meal schedules. The child was not allowed to leave the meal situation for a predetermined time period and was praised by the mother for each bite consumed. The intervention was evaluated using a multiple baseline design across meals (i.e. breakfast and lunch). The results demonstrate an increase in food consumed and decreases in other inappropriate behaviours. The mother continued to implement the treatment successfully during follow-up assessments up to 3 months after the intervention. This is a minimally intrusive intervention in comparison to typical treatments for non-organic food refusal in children with intellectual disabilities.
Record 6Assessment of the influence of background noise on escape-maintained problem behavior and pain behavior in a child with Williams syndrome. O'Reilly MF; Lacey C; Lancioni GE Department of Psychology, National University of Ireland, Dublin, Belfield. [email protected] Journal of applied behavior analysis (United States) Winter 2000, 33 (4) p511-4, ISSN 0021-8855 Languages: ENGLISH Document type: Journal Article
We examined the influence of background noise on levels of problem behavior and pain behavior under functional analysis conditions for a child with a diagnosis of Williams syndrome and hyperacusis. Background noise was associated with increases in escape-maintained problem behavior and increases in pain behavior such as clasping ears and crying. When the child was fitted with earplugs, there were substantial reductions in both problem and pain behavior under the background noise condition.