This project will investigate the development of discourse in adolescents who have mental retardation due to Down syndrome (DS) or fragile X syndrome (FXS). Each syndrome is characterized by impairments in the domains of behavioral functioning that support discourse development (e.g., cognition, receptive language) and, thus, individuals with either syndrome are likely to find discourse to be especially challenging. There are also differences in the behavioral phenotypes of DS and FXS, which may lead to different profiles of discourse problems. There are few data on the discourse skills of individuals with DS or FXS and fewer data still on discourse problems that may be specific to one syndrome or the other. The need for such data is especially acute during the adolescent years. During adolescence, preparation begins for adult roles that demand expertise in discourse. This project will examine discourse in adolescents with DS and adolescents with FXS from a model in which discourse is seen to emerge from the collorative activity of people working toward a common goal. This project has three specific aims. This first is to determine (a) whether discourse development is more severely disrupted in these adolescents than expected from their levels of functioning in domains that support discourse development (i.e., cognition, linguistic ability) and (b) whether it is disrupted differently in the two syndromes.
The second aim i s to (a) identify the ways in which developments of nonverbal cognition, language, social cognition, speech, and hearing contribute to the ability of these adolescents to collaborate effectively and (b) determine whether these domains make different contributions to discourse for DS and FXS.
The third aim i s to determine what syndrome-specific impairments (i.e. psychopathology and auditory memory problems) contribute to discourse collaboration. Five studies will address these aims. In each, participants will engage in a referential task that is designed to highlight differences between the syndromes. Comparisons with younger, typically developing children will determine the extent to which discourse development is disrupted in each syndrome. Measures will be taken of domains in which there are syndrome-shared impairments. Hierarchial multiple regression will be used to examine the contributions of these domains to the discourse collaboration of the DS and FXS groups.

Agency
National Institute of Health (NIH)
Institute
Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD)
Type
Research Project (R01)
Project #
5R01HD024356-13
Application #
6520838
Study Section
Human Development and Aging Subcommittee 3 (HUD)
Program Officer
Hanson, James W
Project Start
1987-09-01
Project End
2004-04-04
Budget Start
2002-07-01
Budget End
2004-04-04
Support Year
13
Fiscal Year
2002
Total Cost
$197,202
Indirect Cost
Name
University of Wisconsin Madison
Department
Pediatrics
Type
Other Domestic Higher Education
DUNS #
161202122
City
Madison
State
WI
Country
United States
Zip Code
53715
Klusek, Jessica; Ruber, Alexis; Roberts, Jane E (2018) Impaired eye contact in the FMR1 premutation is not associated with social anxiety or the broad autism phenotype. Clin Neuropsychol 32:1337-1352
Roberts, Jane E; Ezell, Jordan E; Fairchild, Amanda J et al. (2018) Biobehavioral composite of social aspects of anxiety in young adults with fragile X syndrome contrasted to autism spectrum disorder. Am J Med Genet B Neuropsychiatr Genet 177:665-675
Robinson, Marissa; Klusek, Jessica; Poe, Michele D et al. (2018) The Emergence of Effortful Control in Young Boys With Fragile X Syndrome. Am J Intellect Dev Disabil 123:89-102
Del Hoyo Soriano, Laura; Thurman, Angela John; Harvey, Danielle Jenine et al. (2018) Genetic and maternal predictors of cognitive and behavioral trajectories in females with fragile X syndrome. J Neurodev Disord 10:22
Klusek, Jessica; Porter, Anna; Abbeduto, Leonard et al. (2018) Curvilinear Association Between Language Disfluency and FMR1 CGG Repeat Size Across the Normal, Intermediate, and Premutation Range. Front Genet 9:344
Adlof, Suzanne M; Klusek, Jessica; Hoffmann, Anne et al. (2018) Reading in Children With Fragile X Syndrome: Phonological Awareness and Feasibility of Intervention. Am J Intellect Dev Disabil 123:193-211
Del Hoyo Soriano, Laura; Thurman, Angela John; Abbeduto, Leonard (2018) Specificity: A Phenotypic Comparison of Communication-Relevant Domains Between Youth With Down Syndrome and Fragile X Syndrome. Front Genet 9:424
Klusek, Jessica; Schmidt, Joseph; Fairchild, Amanda J et al. (2017) Altered sensitivity to social gaze in the FMR1 premutation and pragmatic language competence. J Neurodev Disord 9:31
Ashby, Shealyn A; Channell, Marie Moore; Abbeduto, Leonard (2017) Inferential language use by youth with Down syndrome during narration. Res Dev Disabil 71:98-108
Klusek, Jessica; LaFauci, Giuseppe; Adayev, Tatyana et al. (2017) Reduced vagal tone in women with the FMR1 premutation is associated with FMR1 mRNA but not depression or anxiety. J Neurodev Disord 9:16

Showing the most recent 10 out of 66 publications