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Theses and Dissertations

University of South Carolina

Fragile X Syndrome

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Full-Text Articles in Social and Behavioral Sciences

Characterizing Anxiety And Physiological Correlates In Preschool Children With Neurodevelopmental Disabilities, Jordan Ezell Jul 2021

Characterizing Anxiety And Physiological Correlates In Preschool Children With Neurodevelopmental Disabilities, Jordan Ezell

Theses and Dissertations

This dissertation is comprised of two original research manuscripts broadly examining anxiety and potential correlates in young children with neurodevelopmental disabilities. The first manuscript assessed cardiac regulation during an auditory startle paradigm in young children (3-6 years old) with autism spectrum disorder (ASD), fragile x syndrome (FXS), and neurotypical peers. The second manuscript utilized a new measure developed to capture anxiety in individuals with ASD in order to assess the rate of typical and atypical anxiety and potential risk factors (ASD severity, sex, cognitive ability) for anxiety in preschool children with ASD contrasted to neurotypical preschool children. Collectively, these two …


Predicting Change In Autism Symptomatology In Young Children At Risk For Autism Spectrum Disorder: Fragile X Syndrome, Down Syndrome And Non-Syndromic Asd, Kelly Elizabeth Caravella Oct 2019

Predicting Change In Autism Symptomatology In Young Children At Risk For Autism Spectrum Disorder: Fragile X Syndrome, Down Syndrome And Non-Syndromic Asd, Kelly Elizabeth Caravella

Theses and Dissertations

This dissertation is comprised of two manuscripts which examine the longitudinal development of autism symptomatology in young children at risk for developing autism spectrum disorder (ASD); individuals with Fragile x syndrome (FXS) and Down syndrome (DS). The first study is a within group analysis of the longitudinal development of ASD symptomatology in young children with FXS, and how diagnostic stability, language and non-verbal cognitive functioning may predict these trajectories. This paper provides insight into ASD diagnostic stability patterns within FXS, and how symptoms change over time across these groups. The second paper will extend this work by presenting a pilot …


Early Behavioral And Physiological Markers Of Social Anxiety In Fragile X Syndrome, Conner J. Black Oct 2019

Early Behavioral And Physiological Markers Of Social Anxiety In Fragile X Syndrome, Conner J. Black

Theses and Dissertations

Background: Social Anxiety is diagnosed in approximately 10% of neurotypical children. If left untreated, negative outcomes are highly prevalent later in life. Thus, understanding the earliest features of social anxiety can help to mitigate detrimental outcomes. Fragile X Syndrome, which has a high prevalence of social anxiety, is a genetic syndrome which creates a unique opportunity to study the earliest predictors of social anxiety before formal diagnosis. Fragile X Syndrome presents with intellectual disability and an increased prevalence of maladaptive behaviors. The current study utilized a bio-behavioral approach to study the earliest marker of social anxiety in 12- month-old infants …


Prevalence And Predictors Of Attention Deficit Hyperactivity Disorder In Adolescent And Adult Males With Fragile X Syndrome And Autism Spectrum Disorder, Shannon L. O’Connor Jan 2018

Prevalence And Predictors Of Attention Deficit Hyperactivity Disorder In Adolescent And Adult Males With Fragile X Syndrome And Autism Spectrum Disorder, Shannon L. O’Connor

Theses and Dissertations

Individuals with fragile X syndrome (FXS) experience disproportionate rates of diagnoses of attention-deficit/hyperactivity disorder (ADHD) when compared to the general population. Exact prevalence rates and outcomes for individuals with a diagnosis of FXS and ADHD is not well known. The current study assesses rates of ADHD diagnosis in individuals with FXS as well as tests the prediction of a diagnosis of ADHD from levels of autism spectrum disorder (ASD) symptom presentation. The present study included 30 individuals with FXS aged 13-25 and were assessed using a structured diagnostic interview. Interviews were conducted with individual’s parents’ due to low cognitive ability …


Prevalence And Predictors Of Anxiety Disorders In Adolescent And Adult Males With Autism Spectrum Disorder And Fragile X Syndrome, Jordan Ezell Jan 2018

Prevalence And Predictors Of Anxiety Disorders In Adolescent And Adult Males With Autism Spectrum Disorder And Fragile X Syndrome, Jordan Ezell

Theses and Dissertations

disorders are the most prevalent disorders in children and adolescents, affecting approximately 15-20% of individuals under the age of 18 (Salum et al, 2013). Clinical subgroups, like autism spectrum disorder (ASD) and fragile X syndrome (FXS), have an elevated risk of a co-occurring anxiety disorder. Despite the elevated risk of anxiety in these groups, few research studies have investigated the rates and predictors of anxiety disorders in adolescents with these ASD and FXS. In the current study, participants included males with FXS (n=31) or ASD (n=20) aged 16 to 24. Measures included the Children’s Interview for Psychiatric Symptoms-Parent Version (P-ChIPS), …


Emergence Of Stranger Fear In Preschoolers With Fragile X Syndrome And Idiopathic Autism Spectrum Disorder, Jessica Scherr Jan 2017

Emergence Of Stranger Fear In Preschoolers With Fragile X Syndrome And Idiopathic Autism Spectrum Disorder, Jessica Scherr

Theses and Dissertations

This study investigated multiple behavioral indicators of social fear and their relationship to symptoms of autism in preschool boys with atypical and normative development using a stranger approach design. Participants consisted of 101 male preschoolers that were categorized into four groups: boys with fragile X syndrome (FXS; N= 29), FXS with elevated autism symptoms (fxASD; N = 25), idiopathic autism spectrum disorder (iASD; N = 11), and typically developing boys (TD; N = 36). Results indicated specific behavioral responses to a stranger differentiated preschoolers with more severe symptoms of autism (e.g. fxASD and iASD groups) from those with low autism …


Parental Experience Of Divulging A Diagnosis Of Fragile X Syndrome To Their Affected Child, Barbara Alyxandra Athens Jun 2016

Parental Experience Of Divulging A Diagnosis Of Fragile X Syndrome To Their Affected Child, Barbara Alyxandra Athens

Theses and Dissertations

Fragile X syndrome (FXS) is a genetic condition with varied presentation that may include intellectual and learning disabilities, behavioral and learning challenges, and certain physical characteristics. When an individual is affected with FXS, it often leads to complex discussions within the family, including parental disclosure of a FXS diagnosis to a child. This study explored how a FXS diagnosis is communicated between a parent and their child diagnosed with FXS. An online questionnaire was disseminated through FXS organizations and support groups to parents who have at least one child diagnosed with FXS. The online questionnaire was supplemented by eight brief …


Effects Of Cortisol Stress Response Patterns On Autism Related Behaviors In Young Adult Males With Fragile X Syndrome And Autism Spectrum Disorders, Sara M. Matherly Jun 2016

Effects Of Cortisol Stress Response Patterns On Autism Related Behaviors In Young Adult Males With Fragile X Syndrome And Autism Spectrum Disorders, Sara M. Matherly

Theses and Dissertations

Fragile X syndrome (FXS) and autism spectrum disorders (ASD) are characterized by deficits in social interactions (reduced eye contact, topic preoccupation, and social withdrawal) and maladaptive behaviors of anxiety, social withdrawal, and restricted interests (Kau, Tierney, Bukelis, Stump, Kates, Trescher et al., 2004). Prior research with individuals who have FXS has indicated that problem behaviors (i.e. social withdrawal and inattention) were correlated with abnormal activation of the hypothalamus-pituitary-adrenal axis (HPAA; Hessl, et. al., 2002); however, very few studies have investigated physiological patterns and associations with social dysfunction and restricted and repetitive behaviors for individuals with idiopathic ASD. The present study …


Adaptive Skill Trajectories In Infants With Fragile X Syndrome, Kelly Elizabeth Caravella Jan 2016

Adaptive Skill Trajectories In Infants With Fragile X Syndrome, Kelly Elizabeth Caravella

Theses and Dissertations

This study examined longitudinal trajectories of adaptive behavior in infants with fragile X syndrome (FXS), compared to typical development (TD) and infant siblings of children diagnosed with autism (ASIBs). Additional analysis were conducted to examine the differences in trajectories for males and females with FXS, and to identify if a profile of strengths and weaknesses appeared across domains by 24 months in males with FXS. Participants included 76 male infants assessed up to 4 times between 6 and 24 months of age. A sample of 12 females with FXS was also included for the comparative sex analysis. Infants with FXS …