Date on Master's Thesis/Doctoral Dissertation


Document Type

Doctoral Dissertation

Degree Name

Ph. D.


Psychological and Brain Sciences

Degree Program

Clinical Psychology, PhD

Committee Chair

Mervis, Carolyn B.

Committee Co-Chair (if applicable)

Woodruff-Borden, Janet

Committee Member

Woodruff-Borden, Janet

Committee Member

Cashon, Cara H.

Committee Member

Salmon, Paul Gregory

Committee Member

Adelson, Jill L.


Williams syndrome (WS) is a neurodevelopmental disorder caused by a submicroscopic deletion of approximately 25 genes on chromosome 7q11.23 (Hillier et al., 2003). This genotype is associated with a characteristic set of phenotypic features including mild to moderate intellectual disability, a distinctive cognitive profile, facial dysmorphology, common personality traits, cardiovascular problems, and connective tissue disorders (Morris, 2010). In addition, behavioral difficulties such as anxiety symptoms (e.g., Leyfer, Woodruff-Borden, & Mervis, 2009), negative reactivity (e.g., Davies, Udwin, & Howlin, 1998), problems with self-regulation (e.g., Woodruff-Borden, Kistler, Henderson, Crawford, & Mervis, 2010), and sensory modulation difficulties (e.g., John & Mervis, 2010) are common. While anxiety as a behavioral concern for individuals with WS is well-documented, little is known about why these individuals are at an increased risk for this type of problem. Several features of the WS behavioral phenotype have been associated with child anxiety in the general population, specifically negative reactivity (e.g., Lonigan, Phillips, & Hooe, 2003), self-regulation problems (e.g., Eisenberg et al., 2001), and sensory modulation difficulties (e.g., Ben-Sasson, Cermak, Orsmond, Tager-Flusberg, Kadlec, & Carter, 2008). The purpose of this dissertation was to explore the relations of negative reactivity (NR), difficulties with self-regulation (SR), and sensory modulation difficulties (SM) to the increased prevalence of anxiety symptoms in children with WS. Study hypotheses are: (1) The shared variance between NR, SR, and SM will be adequately represented by a single underlying ‘behavioral phenotype’ factor. (2) This factor will share a significant relation with anxiety symptoms in children with WS. Participants were 115 children with WS aged 6 – 10 years. Primary caregivers completed four measures: (1) Children’s Behavior Questionnaire (Rothbart, Ahadi, Hershey, & Fisher, 2001). NR was measured using the Negative Affectivity factor score. (2) Short Sensory Profile (McIntosh, Miller, Shyu, & Dunn, 1999). SM was measured using the Total Sensory Modulation score. (3) Behavior Rating Inventory of Executive Functioning (Gioia, Isquith, Guy, & Kenworthy, 2000). SR was measured using the Behavioral Regulation Index T-score. (4) Child Behavior Checklist 6 – 18 (Achenbach & Rescorla, 2001). Anxiety symptoms were measured using the DSM-IV Anxiety Problems T-score. Exploratory factor analysis and regression analysis were conducted to test the study hypotheses. The results supported both hypotheses: The WS behavioral phenotype features of negative reactivity, difficulties with sensory modulation, and difficulties with self-regulation were adequately represented by one underlying factor; and this factor explained a substantial portion of the variance in anxiety symptoms for children with WS aged 6 – 10 years. Implications for future research and treatment of anxiety in children with WS are discussed.