Neuropsychological Functioning in Preschool-age Children with Sickle Cell Disease: The Role of Illness-related and Psychosocial Factors

Cognitive and academic deficits have been identified in school-aged children with sickle cell disease (SCD). However, there have been very few identified studies that examine neuropsychological functioning in preschool-age children with SCD. It is important to understand effects of SCD from a developmental perspective and to consider the contribution of environmental factors in this at-risk population. Neuropsychological functioning of preschool-age children with SCD and no history of overt stroke (n = 26) was examined across several domains (language, immediate memory/brief attention, visuospatial/visuoconstructional, motor/visuomotor). The mean Full Scale IQ for the sample was 89.0. Performance on the Immediate Memory/ Brief Attention domain was significantly higher than the other domains, although the pattern of performance was relatively consistent, with mean standard scores ranging from 88.0 to 95.0. Disease severity was not significantly related to cognitive functioning in this group of young children with SCD. Socioeconomic status (SES) was significantly correlated with most domain scores and, based on regression analyses, accounted for 18% to 47% of the variance in functioning. Psychosocial factors, such as number of children living in the home and parental stress levels, were negatively associated with Motor/Visuomotor skills, and weekly hours in school/day care was positively associated with language-related skills. Results suggest that, at this young age, psychosocial risk factors appear to be appropriate targets for intervention, with the goal of improving long-term outcome in children with SCD. Further investigations should include comparison to a matched control group.     

Association of social-environmental factors with cognitive function in children with sickle cell disease

The aim of this study was to examine the relationship between cognitive function in pediatric sickle cell disease (SCD) patients and mothers’ reports of social-environmental stress, depressive symptoms, and parenting. A total of 65 children with SCD completed comprehensive neuropsychological testing to assess several domains of cognitive functioning, including general intellectual ability, academic achievement, and executive function. Mothers reported on demographics, social-environmental stress, depressive symptoms, and parenting. As predicted, children with SCD significantly underperformed relative to normative data on measures of cognitive function. Associations between maternal social-environmental stress, maternal depressive symptoms, and parenting were mixed. The results show partial support for the hypothesis that greater stress and depressive symptoms and less positive parenting are associated with poorer cognitive function in children with SCD. Linear regression analyses showed that maternal financial stress was the strongest predictor across all domains of cognitive function. The findings replicate and extend past research, reaffirming that children with SCD are at risk for cognitive impairment across multiple domains. Additionally, social-environmental stress, particularly financial strain, is linked to mothers’ depressive symptoms and parenting behaviors as well as children’s cognitive function. Future studies using direct observations of parenting behaviors are needed. These findings, along with recent research on parenting interventions, may inform the development of concrete, teachable parenting and coping skills to improve cognitive functioning in children with SCD.     

Executive functioning and health-related quality of life in pediatric sickle cell disease

Research consistently indicates that children with sickle cell disease (SCD) face multiple risk factors for neurocognitive impairment. Despite this, no empirical research to date has examined the impact of neurocognitive functioning on quality of life for this pediatric group. Thus, the current study aims to examine the relationship between executive functioning and quality of life in a sample of children with SCD and further explore psychosocial and family/caregiver resources as moderators of this relationship. A total of 45 children with SCD aged 8 to 16 years and their caregivers completed measures of quality of life, behavioral ratings of executive functioning, and psychosocial functioning. Hierarchical linear regression models were utilized to determine the impact of executive functioning on quality of life and further test the interaction effects of proposed moderating variables. Controlling for age, pain, and socioeconomic status (SES), executive functioning was found to significantly predict child- and parent-reported quality of life among youth with SCD. Psychosocial resources of the primary caregiver or family was not found to moderate the relationship between executive functioning and quality of life. These results provide the first empirical evidence that lower executive skills negatively predict quality of life for children with SCD, supporting clinical and research efforts which aim to establish efficacious interventions that target cognitive decrements within this pediatric population.     

Association between biological markers of sickle cell disease and cognitive functioning amongst Cameroonian children

Background: Some of the major complications of sickle cell disease (SCD) occur in the brain and apart from overt stroke, patients also present with cognitive impairments. We sought to evaluate the prevalence of cognitive deficits as well as their biological predicting factors in young SCD patients in Cameroon.

Methods: The cognitive performances of Cameroonian SCD young patients were evaluated using a neuropsychological test battery assessing four domains of cognitive functioning (executive functions, attention, memory, and sensory-motor skills) previously adapted and normalized on healthy subjects in Yaoundé.

Findings: Up to 37.5% of the 96 SCD patients aged 6 to 24 years (M?=?13.5, SD?=?4.9) had mild-to-severe cognitive deficits. The cognitive deficits tend to increase with age. There was a significant effect of SCD on executive functions and attention, whereas SCD patients performed as well as controls on memory and sensory-motor skills tests. Structural equation models showed a significant association between (a) severe anemia and lower executive functioning, (b) low fetal hemoglobin levels and lower executive functioning and attention, (c) history of cerebrovascular accidents and lower performances in executive functioning, sensory-motor skills, and memory, (d) pathological electroencephalogram and lower attention, and (e) abnormal Transcranial Doppler and lower memory.

Conclusion: SCD patients in Cameroon presented a very high prevalence of cognitive deficits, with a specific impairment of executive functions and attention. Routine neuropsychological evaluation for early detection of cognitive deficits in SCD patients could represent a cost-effective tool to implement in resource-limited contexts such as in sub-Saharan Africa.     

Brief report: Relationship between performance testing and parent report of attention and executive functioning profiles in children following perinatal arterial ischemic stroke

Children with perinatal arterial ischemic stroke (PAIS) have increased rates of attention and executive functioning (EF) weaknesses. Research in other pediatric disorders has documented poor consistency between parent report of these skills and performance-based measures. We compared these data sources in children with PAIS. Forty full-term (≥37 weeks) children ages 3–16 (median = 7.2 years; 58% male) with PAIS completed neuropsychological testing and composite scores were created for seven attention and EF domains (Processing Speed; Attention; Working Memory; Verbal Retrieval; Inhibitory Control; Flexibility/Shifting; Planning). Parents completed “real-world” functioning questionnaires (ADHD Rating Scale-IV, BRIEF). Correlational analysis were used to compare parent and performance measures. Correlations between ADHD Rating Scale-IV scores and the performance-based Attention and Inhibition composite scores were nonsignificant. Significant negative correlations were found between the BRIEF GEC and performance-based Verbal Retrieval and Processing Speed composites, but remaining GEC/composite comparisons were nonsignificant. Analyses between parent report BRIEF index scores and the corresponding performance-based domain identified one significant negative correlation between the BRIEF Working Memory Index and the Working Memory composite score. While children with PAIS demonstrate difficulties in attention and EF on both parent report and performance measures, little significance was found in comparisons of these two types of measures. There may be several explanations for this dissociation: measures assessing different aspects of the same underlying construct; performance-based measures lacking ecological validity; and parents underestimating/underreporting their child’s deficits. Thus, multiple sources of informant and performance data are necessary to make more accurate conclusions about functioning in these domains.     

The roles of different executive functioning skills in young children’s mental computation and applied mathematical problem-solving

This study investigated the relationships of four executive functioning skills (including verbal working memory, spatial working memory, inhibitory control, and cognitive flexibility) with young children’s mental computation and applied mathematical problem-solving. Two hundred and twenty-five Chinese kindergarteners were tested with a battery of general cognitive, executive functioning and mathematics skills. Results showed that when children’s age, gender, non-verbal intelligence, and listening comprehension skills were controlled, verbal working memory and cognitive flexibility were significant correlates of mental computation, whereas verbal working memory, spatial working memory, and cognitive flexibility were significant correlates of applied mathematical problem-solving. Inhibitory control was not significantly associated with the two domains of mathematics under investigation. The findings highlight the differential roles of different executive functioning skills in early mathematical skills and offer practical implication for helping young children in learning complex mathematical skills.     

Parent-reported executive functioning in young children treated for cancer

It is well known that children treated for cancer are at risk for cognitive and functional impairments. Such research is largely based on studies of late effects in school-aged or older children. However, far less is known about executive function weaknesses in preschool-aged children treated for cancer. Thus, the aim of this study was to examine executive functioning in a clinically referred sample of young oncology patients, and its association with broader domains of functioning. Data from 61 young children with cancer, who were referred for clinical cognitive evaluations, were abstracted and included in this study. Patients were 5.00 years of age (SD = 0.72) at assessment, 54.1% male, and two-thirds (63.9%) had been treated for brain tumors. Most executive functions were significantly discrepant from the mean, with 47.5% of preschoolers having parent-reported working memory concerns within the clinically significant range. There were no differences in executive functioning based on diagnosis or treatment status. Parent-reported executive functioning was strongly correlated with global intelligence and adaptive functioning, with some indices also associated with nonverbal problem solving and pre-academic skills. Ultimately, results indicate the presence of emerging weaknesses in executive functioning in young children with cancer, and add to a growing body of literature highlighting the potential cognitive and behavioral risks associated with a cancer diagnosis in early childhood.     

Psychometric Properties of the French Version of the Pictorial Scale of Perceived Movement Skill Competence for Young Children (PMSC)

ObjectiveThe objective of this study was to examine the psychometric properties of a French version of the pictorial scale of Perceived Movement Skill Competence for Young Children (PMSC), which is aligned with the third version of the Test of Gross Motor Development.MethodA sample of 219 French-speaking Canadian children (5 to 12 years old) participated in this study.ResultsResults supported the factor validity and reliability of a 13-item version of the PMSC encompassing two factors (i.e., ball skills and locomotor skills). Subsequent analyses supported the weak, partial strong, and partial strict invariance of responses to the PMSC as a function of sex. Additionally, analyses revealed that boys displayed significantly higher perceived ball skill competence than girls. Results also supported a lack of differential item functioning (DIF) and latent mean differences as a function of body mass index and physical activity/sport involvement, but revealed evidence of DIF and latent mean differences as a function of age. More precisely, these results show that older children displayed significantly: (a) higher scores on the sliding item and lower scores on the kicking item relative to younger children; and (b) lower scores on perceived locomotor skills competence than younger children.ConclusionOverall, results suggest that the French version of the PMSC has acceptable psychometric properties and can be confidently used in research or practice to assess children's perceived movement skill competence.     

Links between mothers' and children's social competence and associations with maternal adjustment.

Extended previous research on family factors related to children's social competence by examining links between 3 domains of maternal social competence (social skills, social frames, network size) and 3 domains of children's social competence (social skills, peer-rated social preference, and network size) in a sample of 78 mothers and their kindergarten children (42 girls, 36 boys). The mediational role of maternal social competence in the association between mothers' depression and interpersonal sensitivity symptoms and children's social competence was also tested. Results supported associations between all 3 domains of mothers' and children's social competence, with maternal social skills the most frequent predictor of children's social competence. Gender differences suggested that maternal social competence was more strongly related to sons' peer acceptance and daughters' social skills. A mediational model received preliminary support for girls, with maternal psychological symptoms related to girls' social competence via maternal social skills. Implications include the need for continued study of maternal social competence as a family factor related to children's peer functioning and the development of family-based interventions for young children experiencing peer problems.     

Cognitive and developmental influences in visual-motor integration skills in young children

Measures of visual-motor integration skills continue to be widely used in psychological assessments with children. However, the construct validity of many visual-motor integration measures remains unclear. In this study, we investigated the relative contributions of maturation and cognitive skills to the development of visual-motor integration skills in young children (N = 856). We used a block regression analysis to determine the contribution of maturation, as indicated by age, followed by broad cognitive factors (Study 1) and subsequently by individual subtests in verbal and nonverbal domains subsumed under each factor (Study 2) in explaining score variance of the Bender Visual-Motor Gestalt Test (2nd ed.; BG-II; Brannigan & Decker, 2003) Copy and Recall scores in children between the ages of 4 and 7 years. Results confirm that maturation accounted for a large proportion of variance in both BG-II Copy and Recall performance, above which Stanford-Binet Intelligence Scale (5th ed.; SB-5; Roid, 2003) Quantitative Reasoning and Fluid Reasoning factors significantly contributed to visual-motor integration performance for the Copy phase, and SB-5 Quantitative Reasoning and Visual-Spatial factors accounted for a significant amount of variance for the Recall phase. Additionally, nonverbal domains were more related to visual-motor performance than verbal domains. Results from this study are interpreted to suggest nonverbal reasoning and visual-spatial attention are important contributing factors to visual-motor integration, as measured by the BG-II. Developmental implications of visual-motor integration skills, nonverbal problem solving, and mathematical competence are discussed.     

Behavioral and cognitive-affective features of stuttering in preschool-age children: Regression and exploratory cluster analyses

PurposeThe purpose of this study was to investigate associations among behavioral and cognitive-affective features of stuttering in preschool-age children who stutter, and the extent to which participants may or may not cluster together based on multiple indices of stuttering.MethodsParticipants were 296 preschool-age children who stutter (mean age 47.9 months). Correlation and regression analyses, as well as k-means cluster analyses were conducted between and among several indices of stuttering: frequency of stuttering- and non-stuttering-like disfluencies (SLDs and NSLDs), ratios of repetitions and prolongations/blocks out of total number of SLDs, associated nonspeech behaviors, duration of stuttering events, KiddyCAT scores (Vanryckeghem & Brutten, 2007), and a TOCS parent-rated scale (Gillam et al., 2009).ResultsFor preschool-age children who stutter, most indices of overt stuttering behaviors were intercorrelated (e.g., more SLDs were associated with higher ratio of repetitions). Self-reported KiddyCAT scores (Vanryckeghem & Brutten, 2007) were largely not significantly associated with stuttering. Cluster analyses yielded two participant groupings: a larger group with less prominent stuttering features and a smaller group with more prominent features.ConclusionsThis study contributes to an increasingly comprehensive and nuanced understanding of the heterogeneous features of stuttering and their development in preschool-age children. Findings show strong intercorrelations between measures of stuttering behaviors, but more tenuous relationships between behaviors and cognitive-affective reactions to stuttering. Exploration of clusters of characteristics within this population revealed potential opportunities for future research.     

Utility of the Behavior Rating Inventory of Executive Function – Preschool version (BRIEF-P) in young children with epilepsy

Executive functioning deficits are prominent in children with epilepsy. Although instruments, such as the Behavior Rating Inventory of Executive Function (BRIEF), are useful in detecting executive dysfunction in school-age children with epilepsy, little data are available for younger children. The present study evaluates the ability of the Behavior Rating Inventory of Executive Function – Preschool Version (BRIEF-P) to detect executive dysfunction in preschool-age children with epilepsy. Parents of 51 clinically referred children with epilepsy (age: M = 1.99 years, SD = 1.29 years, range = 2–5 years) completed the BRIEF-P. Using a cutoff t score of ≥65 as the threshold for impairment, the BRIEF-P’s ability to detect executive dysfunction within this clinical population was established. Additionally, correlational analyses were used to assess the relations between epilepsy severity factors and BRIEF-P indices. Epilepsy severity variables that were significantly related to BRIEF-P indices were entered into a linear regression model to explore their predictive ability. Emergent metacognition (emergent metacognition index [EMI]; 59%) and the global executive composite (43%) were the most frequently elevated indices. The most commonly elevated subscales were working memory (65%), inhibition (37%), and planning/organization (35%). Age of seizure onset, seizure frequency, and number of antiepileptic drugs were not significantly correlated with BRIEF-P indices. However, children with lower intellectual ability were rated as having greater executive dysfunction, specifically with EMI (r = ?.30). Still, intellectual functioning only accounted for a small percentage (9%) of the variance in EMI scores. The current pilot study demonstrates that the BRIEF-P shows promise in identifying executive dysfunction in preschool-age children with epilepsy.     

Use of Gilliam Asperger's disorder scale in differentiating high and low functioning autism and ADHD

Little is known about the validity of Gilliam Asperger's Disorder Scale (GADS), although it is widely used. This study of 199 children with high functioning autism or Asperger's disorder, 195 with low functioning autism, and 83 with attention deficit hyperactivity disorder (ADHD) showed high classification accuracy (autism vs. ADHD) for clinicians' GADS Quotients (92%), and somewhat lower accuracy (77%) for parents' Quotients. Both children with high and low functioning autism had clinicians' Quotients (M=99 and 101, respectively) similar to the Asperger's Disorder mean of 100 for the GADS normative sample. Children with high functioning autism scored significantly higher on the cognitive patterns subscale than children with low functioning autism, and the latter had higher scores on the remaining subscales: social interaction, restricted patterns of behavior, and pragmatic skills. Using the clinicians' Quotient and Cognitive Patterns score, 70% of children were correctly identified as having high or low functioning autism or ADHD.     

Intellectual and adaptive functioning in Sturge-Weber Syndrome

The present study examined the intellectual and adaptive functioning in a sample of children and young adults with Sturge-Weber Syndrome (SWS). A total of 80 research participants from a SWS study database underwent full neurological evaluation as part of their participation or concurrent medical care. Twenty-nine of the participants received neuropsychological evaluations. Analyses indicated no significant demographic or neurological differences between those who did and did not receive neuropsychological evaluations. Overall, the neuropsychological evaluation sample displayed significantly lower functioning relative to published normative data across domains of intellectual and adaptive functioning. Thirty-two percent of the sample displayed impaired performance (standard score ≤ 75) in intellectual functioning and 58% displayed impaired performance in adaptive functioning. Hemiparesis status independently predicted overall adaptive functioning while seizure frequency independently predicted overall intellectual functioning. Younger participants displayed significantly higher (more intact) ratings in adaptive functioning compared to older participants, specifically in overall adaptive functioning, motor skills, and community living skills. A composite measure of neurological status (SWS-NRS) incorporating seizure and hemiparesis status effectively distinguished between individuals with impaired or nonimpaired adaptive and intellectual functioning and showed promise as a screening method for identifying individuals with more involved intellectual and/or adaptive needs.     

Hypoxia and Inflammation in Children with Sickle Cell Disease: Implications for Hippocampal Functioning and Episodic Memory

Children with sickle cell disease (SCD) suffer from systemic processes (e.g., chronic anemia, recurrent hypoxic-ischemic events, chronic inflammation) that have been associated with neurocognitive impairment in a range of clinical populations, but which have been largely understudied in relation to specific domains of cognitive functioning in children with SCD. This review focuses on episodic memory, as the hippocampus may be especially vulnerable to the systemic processes associated with SCD. The first part of the paper outlines the pathophysiology of SCD and briefly reviews the extant literature on academic and cognitive functioning in children with SCD, emphasizing the dearth of research on episodic memory. Next, the complex systemic processes of hypoxia and inflammation associated with SCD are reviewed, along with research that has associated these processes with hippocampal damage and memory impairment. The paper concludes with suggestions for future research that are informed, in part, by the literature on developmental amnesia.     

School competence and emotional/behavioral problems among Norwegian school children as rated by teachers on the Teacher Report Form

Of 1,409 eligible children aged 6-13 years in grades 1 to 7 who were randomly selected from a national sample of Norwegian schools, 858 participated in the present study (60.9%). The sample was stratified by school centrality, region and size of grade cohort. The teachers assessed their children's academic performance, adaptive school functioning, and levels of emotional/behavioral problems using the 2001 version of the Teacher Report Form (TRF). Only one child was randomly selected from each grade cohort. Girls had significantly higher scores than boys in the Working Hard, Appropriate Behavior, Learning, and Total Adaptive Functioning domains. For girls, only the Working Hard domain was of medium effect size. While boys had significantly higher scores than girls on Attention, Thought Problems, Rule-Breaking, Aggression, Externalizing Problems and Total problems, only Attention Problems showed a medium effect size. Significant sex by age interaction effects were also found for Rule-Breaking, Externalizing, Internalizing, Anxious-Depressed and Total Problems. In all these comparisons, 10-13-year-old boys had significantly higher scores than 6-9-year olds, while girls had similar problem levels across age groups. Our mean Total Problems score (17.2) was lower than the grand mean (21.6) reported in a multi-country comparison but higher than in another Norwegian large-scale survey. Overall, our findings indicate that teachers in Scandinavia report, just as do parents, relatively low levels of emotional/behavioral problems among school-aged children.     

Parental Problem-Solving Abilities and the Association of Sickle Cell Disease Complications with Health-Related Quality of Life for School-Age Children

Children with sickle cell disease (SCD) are at risk for poor health-related quality of life (HRQOL). The current analysis sought to explore parent problem-solving abilities/skills as a moderator between SCD complications and HRQOL to evaluate applicability to pediatric SCD. At baseline, 83 children ages 6–12 years and their primary caregiver completed measures of child HRQOL. Primary caregivers also completed a measure of social problem-solving. A SCD complications score was computed from medical record review. Parent problem-solving abilities significantly moderated the association of SCD complications with child self-report psychosocial HRQOL (p = .006). SCD complications had a direct effect on parent proxy physical and psychosocial child HRQOL. Enhancing parent problem-solving abilities may be one approach to improve HRQOL for children with high SCD complications; however, modification of parent perceptions of HRQOL may require direct intervention to improve knowledge and skills involved in disease management.     

Incidence of Specific Absolute Neurocognitive Impairment in Globally Intact Children with Histories of Early Severe Deprivation

Postnatal deprivation is associated with neurocognitive delay/dysfunction. Although “catch up” in global cognition following adoption has been reported, this study examined the incidence of specific absolute impairment in adopted children with intact global cognitive functioning. Eighty-five children (38 males, mean age = 112.8, SD = 30.3 months; range 61–209 months) raised from birth in orphanages underwent comprehensive neuropsychological evaluation. Fifty-four were deemed globally intact (IQ > 85). Of those deemed globally intact, 46% evidenced absolute impairment in at least one domain of functioning. Duration of stay in the orphanage was directly associated with incidence of impairment and number of domains affected. A substantial proportion of participants evidenced persistent, absolute impairment in one or more domains of neurocognitive function despite integrity of basic intellectual functions.     

Incidence of specific absolute neurocognitive impairment in globally intact children with histories of early severe deprivation

Postnatal deprivation is associated with neurocognitive delay/dysfunction. Although "catch up" in global cognition following adoption has been reported, this study examined the incidence of specific absolute impairment in adopted children with intact global cognitive functioning. Eighty-five children (38 males, mean age = 112.8, SD = 30.3 months; range 61-209 months) raised from birth in orphanages underwent comprehensive neuropsychological evaluation. Fifty-four were deemed globally intact (IQ > 85). Of those deemed globally intact, 46% evidenced absolute impairment in at least one domain of functioning. Duration of stay in the orphanage was directly associated with incidence of impairment and number of domains affected. A substantial proportion of participants evidenced persistent, absolute impairment in one or more domains of neurocognitive function despite integrity of basic intellectual functions.     

Perceptuomotor functioning in preschool children with symptoms of attention deficit hyperactivity disorder

Given limited research on perceptual-motor functioning of preschool children with symptoms of ADHD, the purpose of this study was to compare the fine motor, gross motor, visuomotor, and oral-motor functioning of 49 4- to 6-yr.-old children with symptoms of Attention Deficit Hyperactivity Disorder and 48 typically developing children. Analysis showed scores of the ADHD group were significantly lower than those of the control group on all perceptuomotor measures. In addition, scores on all measures yielded significant correlations with scores on the Hyperactive-Inattentive scale of the Preschool Behavior Questionnaire. Regression analyses indicated that the scores of gross motor and visuomotor functioning were significant predictors of group classification (with and without ADHD). These findings extend the well documented findings of perceptuomotor deficits among school-age children with ADHD into the preschool period and emphasize the importance of early assessment and treatment of these deficits in young children with symptoms of ADHD.