Stephen R Hooper

University of North Carolina at Chapel Hill | UNC

The unusually broad heterogeneity of physical, developmental, cognitive, and behavioral presentations among children diagnosed with 22q11.2 deletion syndrome (22q11DS) renders generalized predictions of outcomes and interventions impossible. Rather, each child’s unique array of strengths and weaknesses must be considered and periodically updated as...

Objectives To describe the presence and persistence of neurological and neuropsychological sequelae among children with acquired Zika virus infection and assess whether those sequelae were more common in children infected with Zika virus compared to uninfected children. Methods We conducted a prospective cohort study of children with and without Z...

Objective To compare specific attention functions for school-age children with chronic kidney disease (CKD) to those of a typically developing control group. Methods A cross-sectional study examined attention dimensions for children and adolescents with CKD ( n = 30) in comparison to a typically developing control group ( n = 41). The CKD group co...

Background: There is currently a dearth of research on the neural framework of writing tasks in children, as measured by neuroimaging techniques. Objective: This paper provides an overview of the current literature examining the neurological underpinnings of written expression in children. Design: Using a scoping review approach, with thorough...

Executive functions are a set of cognitive processes we use to act on information, manage resources, and plan and monitor our own behaviour, all with the aim of achieving an end goal. These are skills that develop from infancy. While ‘reading’ has been extensively studied in psychology literature, ‘writing’ has been somewhat neglected, despite a la...

Background: Returning to learn following a concussion is the process of managing a student's recovery during the school day by implementation of academic supports with varying intensity. Due to a lack of consensus or even guidance on Return to Learn, this paper set out to establish cross discipline consensus on some essential elements of Return to...

Traumatic Brain Injury (TBI) is a special education eligibility category under the Individuals with Disabilities Education Act (IDEA). Unlike other special education categories (e.g., autism, specific learning disabilities), relatively few students with TBI are identified for special education nationwide compared to the known prevalence of TBI. Dis...

22q11.2 Deletion Syndrome (22qDS) is a neurogenetic disorder resulting in cognitive deficits and hypogyrification, but relationships between these processes have not been established. 22qDS youth and healthy controls (HC) were administered a battery of cognitive tasks. Gyrification measurements were extracted from structural T1 scans using Freesurf...

Background 22q11.2 Deletion Syndrome (22q11.2DS) is associated with high risk for developing schizophrenia in adulthood while Attention Deficit Hyperactivity Disorder (ADHD) constitutes the most frequent diagnosis in childhood. Individuals with 22q11.2DS show marked inattention symptoms. Interestingly, schizophrenia is also characterized by attenti...

Schizophrenia is a neurodevelopmental disorder that affects up to 1% of the general population. Various genes show associations with schizophrenia and a very weak nominal association with the tight junction protein, claudin-5, has previously been identified. Claudin-5 is expressed in endothelial cells forming part of the blood-brain barrier (BBB)....

This paper examines family experiences with the efficiency of ASD diagnosis. Children were age 8 or younger with ASD (n = 450). Outcomes were delay from first parent concern to diagnosis, shifting diagnoses, and being told child did not have ASD. Predictors were screening, travel distance, and problems finding providers. Logit models were used to e...

Background: It is vital to engage in systematic screening to identify and serve children who may have sustained an acquired brain injury (ABI) - either traumatic or non-traumatic, so they can be successfully transitioned between environments and life stages. This is particularly important for children and adolescents given the impact an ABI can ha...

Little is known about the psychosocial profiles of parents who have a child with an undiagnosed chronic illness. The National Institutes of Health Undiagnosed Diseases Network (UDN) evaluates individuals with intractable medical findings, with the objective of discovering the underlying diagnosis. We report on the psychosocial profiles of 50 parent...

Background: Prenatal alcohol exposure can affect neurodevelopment, but few studies have examined associations with autism spectrum disorder (ASD). Methods: We assessed the association between maternal alcohol use and ASD in the Study to Explore Early Development, a multi-site case-control study of children born between September 2003 and August...

Impairments in executive function, such as working memory, are almost universal in children with chromosome 22q11.2 deletion syndrome. Delineating the neural underpinnings of these functions would enhance understanding of these impairments. In this study, children and adolescents with 22q11 deletion syndrome were compared with healthy control parti...

Objectives: To focus attention on building statewide capacity to support students with mild traumatic brain injury (mTBI)/concussion. Method: Consensus-building process with a multidisciplinary group of clinicians, researchers, policy makers, and state Department of Education personnel. Results: The white paper presents the group's consensus o...

Background: Posttraumatic stress disorder (PTSD) is considered a disorder of recovery where individuals fail to learn and retain extinction of the traumatic fear response. In maltreated youth, PTSD is common, chronic, and associated with co-morbidity. Studies of extinction-related structural volumes (amygdala, hippocampus, anterior cingulate cortex...

Children with chromosome 22q11.2 deletion syndrome (22q11DS) often have deficits in social cognition and social skills that contribute to poor adaptive functioning. These deficits may be of relevance to the later occurrence of serious psychiatric illnesses such as schizophrenia. Yet, there are no evidence-based interventions to improve social cogni...

This issue brief provides an overview of the "state of the state" for traumatic brain injury (TBI) issues and challenges in North Carolina. A previous issue of the North Carolina Medical Journal discussed this topic approximately 14 years ago, and this issue brief showcases changes and advances since that time. Collectively, articles in the current...

This issue brief provides an overview of the "state of the state" for traumatic brain injury (TBI) issues and challenges in North Carolina. A previous issue of the North Carolina Medical Journal discussed this topic approximately 14 years ago, and this issue brief showcases changes and advances since that time. Collectively, articles in the current...

This longitudinal study was conducted to determine (a) the rate of co-occurrence of reading disabilities (RDs) in a writing disability (WD) population of students followed from first grade to fourth grade and (b) the cognitive burden that is assumed by having a WD and a RD (WD + RD). The sample included 137 first-grade students from a single school...

The objective of this study was to determine the relationship between a history of dyslexia and childhood physical abuse in a large population-based epidemiological sample. It was hypothesized that the prevalence of dyslexia would be significantly higher in individuals who reported a childhood history of physical abuse in comparison to those who di...

Objective: The purpose of this study was to evaluate atomoxetine treatment effects in attention-deficit/hyperactivity disorder (ADHD-only), attention-deficit/hyperactivity disorder with comorbid dyslexia (ADHD+D), or dyslexia only on ADHD core symptoms and on sluggish cognitive tempo (SCT), working memory, life performance, and self-concept. Meth...

Background: Children with 22q11.2 deletion syndrome (22q11DS) are at risk for social-behavioural and neurocognitive sequelae throughout development. The current study examined the impact of family environmental characteristics on social-behavioural and cognitive outcomes in this paediatric population. Method: Guardians of children with 22q11DS w...

CKD has been linked with cognitive deficits and affective disorders in multiple studies. Analysis of structural and functional neuroimaging in adults and children with kidney disease may provide additional important insights into the pathobiology of this relationship. This paper comprehensively reviews neuroimaging studies in both children and adul...

Velocardiofacial and DiGeorge syndromes, also known as 22q11.2 deletion syndrome (22q11DS), are congenital-anomaly disorders caused by a de novo hemizygous 22q11.2 deletion mediated by meiotic nonallelic homologous recombination events between low-copy repeats, also known as segmental duplications. Although previous studies exist, each was of small...

Chromosome 22q11.2 deletion syndrome (22q11DS) is the most common microdeletion syndrome in humans. It is typified by highly variable symptoms, which might be explained by epigenetic regulation of genes in the interval. Using computational algorithms, our laboratory previously predicted that DiGeorge critical region 6 (DGCR6), which lies within the...

Translational Psychiatry explores the more translational area between the research in neuroscience and conceptually novel treatments

Although distinctive neuropsychological impairments have been delineated in children with chromosome 22q11 deletion syndrome (22q11DS), social skills and social cognition remain less well-characterised. To examine social skills and social cognition and their relationship with neuropsychological function/behaviour and psychiatric diagnoses in childr...

Early identification of autism spectrum disorders has been demonstrated to result in material benefits to children, their families, and society by reducing problem behaviors, improving academic achievement and school outcomes, and increasing social participation. Early intervention also reduces the costs and associated morbidity of autism spectrum...

Working memory problems have been targeted as core deficits in individuals with Fragile X syndrome (FXS); however, there have been few studies that have examined working memory in young boys with FXS, and even fewer studies that have studied the working memory performance of young boys with FXS across different degrees of complexity. The purpose of...

This book provides a detailed account of intellectual, other neuropsychological and behavioral manifestations of general pediatric diseases. The conditions discussed include the whole range of pediatric diseases - genetic syndromes, other congenital conditions, metabolic, endocrine, gastrointestinal, infectious, immunologic, toxic, trauma, and neop...

Although symptoms of sleepiness and fatigue are common in adults with chronic kidney disease (CKD), little is known about the prevalence of these symptoms in children with CKD. Cross-sectional analysis within a cohort study. We describe the frequency and severity of sleep problems and fatigue and assess the extent of their association with measured...

Our chapter in the earlier editions of the Handbook (Tramontana & Hooper, 1989; 1997) opened with a series of questions.

Attention deficit hyperactivity disorder (ADHD) and learning disabilities (LD) are the most commonly used diagnoses for children who experience academic and behavioral difficulties. Not only do questions concerning ADHD and LD comprise the majority of referrals made to psychologists, psychiatrists, and other professionals, but many children diagnos...

Sustained attention and response inhibition were examined in boys with full mutation fragile X syndrome (FXS) using adapted visual and auditory continuous performance tests (CPTs). Only 61% of 56 boys with visual CPT data and 54% of 52 boys with auditory data were able to demonstrate sufficient understanding to complete the visual and auditory CPTs...

The purpose of the study was to explore associations between neurocognitive function and chronic kidney disease (CKD)-related clinical characteristics. Twenty-nine children, ages 7 to 19 years, with an estimated creatinine clearance (eCrCl) of 4-89 ml/min per 1.73 m2 body surface area were enrolled. Intellectual function (IQ), memory, and attention...

Identifying many of the diagnostic criteria for anxiety and depression in individuals with intellectual disability (ID) can be challenging because they may be unable to recognize and communicate their emotional experiences accurately. The purpose of this study is to identify behavioural equivalents of anxiety in children with fragile X syndrome (FX...

Parent- and teacher-report of attention-deficit/hyperactivity disorder (ADHD) symptoms were examined using problem behavior and DSM-IV symptom inventory questionnaires for 63 children with full mutation fragile X syndrome (FXS) and 56 children without disabilities matched on mental age (MA). Prevalence rates of ADHD symptoms varied depending on typ...

SYNOPSIS Objective. African American children exposed to multiple social risk factors during early childhood often experience academic difficulties, so identifica-tion of protective factors is important. Design. Academic and school behavior trajectories from kindergarten through third grade were studied among 75 African American children who have b...

This article reviews an emergent area of traumatic brain injury (TBI) literature; namely, developmental outcomes of TBI sustained during the early childhood and preschool period. The developmental time period from birth through age 5 years is one of significant growth and maturity, particularly in the neurological development of the child. An injur...

This study was conducted to assess the perceptions of school psychologists regarding myths and misconceptions pertaining to traumatic brain injury (TBI). A sample of 304 school psychologists in the state of North Carolina was surveyed on 11 common myths and misconceptions about TBI. Results indicated that this group performed significantly better t...

The academic achievement of boys with fragile X syndrome and the relation between several predictive factors and academic performance are reported. Boys with fragile X syndrome displayed significant deficits in all academic skill areas. Relative strengths were observed in general knowledge, reflecting the ability to integrate experiential informati...

This study examines the developmental changes in nonverbal intellectual functioning evident in males with fragile X syndrome (FXS) measured by the Leiter International Performance Scales-Revised (Leiter-R). The Leiter-R provides both IQ scores and associated growth scores which permit the examination of both age-based IQ scores and overall intellec...

The authors examined (a) the extent to which kindergarten estimates of core language functions predicted teacher ratings of behavior problems in each of the child's first 4 years of elementary school and (b) the ability of core language measures to predict concurrent behavior problems at each of the early elementary school grades studied. Participa...

Several lines of evidence implicate dysfunction in brain energy production as a key component of bipolar disorder. In particular, elevated brain lactate levels observed in this condition suggest a shift from aerobic to anaerobic metabolism, possibly as a result of mitochondrial abnormalities. Most prior imaging studies of brain metabolites were per...