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  • Cattell-Horn-Carroll Theory...
    Aguirre-Kolb, Christina

    01/2021
    Dissertation

    Children with a full mutation of Fragile X Syndrome (FXS) have a similar genetic make-up and yield consistently severe cognitive disabilities (CDC, 2018). The goal of this research is to ascertain if a cognitive profile exists for these FXS individuals that would enhance the tailoring of the delivery of services for this condition. To advance the development of a cognitive profile the evaluation of cognitive abilities needs to occur within the structure of cognitive theory, such as the evidenced-based, Cattell-Horn-Carroll (CHC) theory. This theory provides the theoretical context from which methodological refinements can be made to detect the relative strengths and weaknesses of FXS individuals. This methodological change is the application of the z-score deviation method (Hessl et al.,2009 & Sansone et al.,2014) to the raw scores of Woodcock-Johnson IV (WJ-IV) Tests of Cognitive Ability for each participant. Due to COVID-19, the assessments were conducted via video conferencing on 16 children with FXS, a novel and successful assessment using the WJ-IV. Results indicated that the mean scores for children with FXS were significantly lower than the average scores on each of the cognitive domains of the third stratum of the CHC profile compared to the norm means for children with developmental delays. A cognitive profile in the children with FXS was evident by the consistent rank orderings of the subtests across individuals. This was demonstrated graphically and statistically, revealing consistent cognitive strengths and weaknesses in this group. The implications for treatment of FXS and future directions of cognitive assessment are discussed.