Neuropsychological Functioning in Children with Medium Chain Acyl Coenzyme A Dehydrogenase Deficiency (MCADD): The Impact of Early Diagnosis and Screening on Outcome

Children with medium chain acyl coenzyme A dehydrogenase deficiency (MCADD) have been reported to be at high risk for neurocognitive deficits. However this has not been systematically studied and little is known about the exact nature of neuropsychological sequelae or of the impact of early diagnosis and screening on outcome. We examined cognitive and adaptive outcome in children with MCADD (N?=?38, age range: 2 years, 2 months – 10 years, 3 months) diagnosed either through a newborn screening program (tandem mass spectrometry/MSMS) or upon clinical presentation. There was no evidence of overall intellectual impairment in either groups but there was some suggestion of poorer verbal and specific executive functioning (i.e., planning) abilities in the unscreened cohorts. Adaptive functioning was relatively intact with the exception of reduced Daily Living Skills in both our screened and unscreened groups. Early diagnosis and greater number of hospitalizations were related to higher verbal, communication, and socialization skills. Overall, our results highlight the importance of early diagnosis and management for children with MCADD.     

Reading and Spelling Disabilities in Children with and Without a History of Early Language Delay: A Neuropsychological and Linguistic Study

Language delay is a frequent antecedent of literacy problems, and both may be linked to phonological impairment. Studies on developmental dyslexia have led to contradictory results due to the heterogeneity of the pathological samples. The present study investigated whether Italian children with dyslexia showed selective phonological processing deficits or more widespread linguistic impairment and whether these deficits were associated with previous language delay. We chose 46 children with specific reading deficits and divided them into two groups based on whether they had language delay (LD) or not (NoLD). LD and NoLD children showed similar, severe deficits in reading and spelling decoding, but only LD children showed a moderate impairment in reading comprehension. LD children were more impaired in phonological working memory and phonological fluency, as well as in semantic fluency, grammatical comprehension, and verbal IQ. These findings indicate the presence of a moderate but widespread linguistic deficit (not limited to phonological processing) in a subset of dyslexic children with previous language delay that does not generalize to all children with reading difficulties.     

Towards a new method for bridging the gap between “smart” infants and “dumb” preschoolers

Based on violation-of-expectation (VoE) paradigms, amazing cognitive competencies have been demonstrated in young infants, which could not be shown in toddlers or even preschoolers. This divergence might as much be caused by different research methods as by discontinuities in development. As looking-time measures are not readily applicable to older children, we suggest a new method that is suitable for children from two years of age onwards. In an empirical examination of this method, 26 children aged 2–7 years learned by trial and error to always find a target picture among a pair of pictures. Each target picture was an impossible version of the non-target picture. After reaching a learning criterion, children had to generalize the learned concept to pictures belonging to a different category. Results showed that even the youngest participants reached the learning criterion and were able to apply what they had learned to another category.     

Identity-based inhibitory processing during focused attention

Recent data suggest that the operating principles governing inhibition of distractor-related activity may deviate from dominant models describing inhibitory processing during selective attention. Here we aimed to gain a better understanding of these data in order to determine whether they actually defy premises of current models. In addition to providing evidence against noninhibitory accounts of the data (see especially Experiment 6), the results support three main novel findings that challenge current theories. First, the data provide evidence that inhibition overpowered excitation from ongoing external input (Experiments 1–4), which suggests that inhibitory control processes are more powerful than current models indicate. Second, negative effects emerged even when targets appeared alone (Experiment 5), which suggests that selection does not play an essential role in triggering inhibitory processing. Third, relatively early distractor-related activity was affected, which supports a role for inhibition prior to action control (Experiment 3). These findings suggest a need to revise current models describing inhibition of distracting information during selective attention.     

Perceptions of Self and Relationship with Parents in Aggressive and Nonaggressive Rejected Children

This study examined the perceptions of self and the attachment relationship to parents in aggressive and nonaggressive rejected children, as compared to children with an average or popular sociometric status. Participants were 216 children from grades 3 and 4. They completed peer nomination measures, the Self-Perception Profile for Children (SPPC), and the Security Scale (mother and father form). Results generally confirmed the assumption that only the nonaggressive rejected children perceive themselves as less competent and worthy as a person than the more popular children. Aggressive rejected children did not report lower feelings of global self-worth or competence, although they did report lower levels of social acceptance. Furthermore, rejected-nonaggressive children perceived the relationship with their father (but not with their mother) as less secure than did the more popular children. Logistic regression analyses suggested that the linkage between felt security with father and rejected-nonaggressive status was mediated by children's self-worth. Implications for attachment theory and for the hypothesized heterogeneity among rejected children are discussed.     

The Prevention of Reading Difficulties

The purpose of this article is to provide practical advice about methods to prevent reading failure that is grounded in the new knowledge we have acquired about reading and learning to read over the past 2 decades. Recent research on reading is used to establish a set of facts about reading and reading growth that is relevant to establishing instructional objectives and methods for the prevention of reading difficulties. Within the context of our current understanding of the reasons many children find it difficult to learn to read, the article also identifies the instructional conditions that need to be in place to prevent the development of reading difficulties in all but a very small proportion of children. The article concludes with a discussion of issues and procedures for the early identification of children who are likely to experience difficulties learning to read.     

Fostering young children's representation, planning, and reflection: A focus in three current early childhood models

Many educators and developmental psychologists have pointed to the development of symbolic thought and representational abilities as a set of fundamental capacities that underlie the development of self-regulation, problem solving, planning, and higher level thought processes. While many early childhood programs value children's development of symbolic capacities, three approaches, High/Scope, Tools of the Mind, and Reggio Emilia, were analyzed in terms of one context from the array of strategies and experiences that characterizes the approach in order to examine the distinctive way that three different preschool programs apply the theoretical construct of representational development to pedagogical strategies.     

New Perspectives on Agency in Early Modern Philosophy

ABSTRACT

This introductory article outlines the themes and aims of this special issue, which offers new perspectives on early modern debates about agency in two ways: First, it recovers writings on agency and liberty that have been widely neglected or that have received insufficient attention, including writings by Anne Conway, Henry More, Ralph Cudworth, William King, Gabrielle Suchon, Elizabeth Berkeley Burnet, Mary Astell, and Anthony Ashley Cooper, the Third Earl of Shaftesbury. Second, it reveals the richness of early modern debates about agency and liberty.     

Predictive model for early math skills based on structural equations

Early math skills are determined by higher cognitive processes that are particularly important for acquiring and developing skills during a child's early education. Such processes could be a critical target for identifying students at risk for math learning difficulties. Few studies have considered the use of a structural equation method to rationalize these relations. Participating in this study were 207 preschool students ages 59 to 72 months, 108 boys and 99 girls. Performance with respect to early math skills, early literacy, general intelligence, working memory, and short‐term memory was assessed. A structural equation model explaining 64.3% of the variance in early math skills was applied. Early literacy exhibited the highest statistical significance (β = 0.443, p < 0.05), followed by intelligence (β = 0.286, p < 0.05), working memory (β = 0.220, p < 0.05), and short‐term memory (β = 0.213, p < 0.05). Correlations between the independent variables were also significant (p < 0.05). According to the results, cognitive variables should be included in remedial intervention programs.     

Concurrent validity of the differential ability scales,second edition with the Mullen Scales of Early Learning in young children with and without neurodevelopmental disorders

Estimates of intelligence in young children with neurodevelopmental disorders are critical for making diagnoses, in characterizing symptoms of disorders, and in predicting future outcomes. The limitations of standardized testing for children with developmental delay or cognitive impairment are well known: Tests do not exist that provide developmentally appropriate material along with norms that extend to the lower reaches of ability. Two commonly used and interchanged instruments are the Mullen Scales of Early Learning (MSEL), a test of developmental level, and the Differential Ability Scales, second edition (DAS-II), a more traditional cognitive test. We evaluated the correspondence of contemporaneous MSEL and the DAS-II scores in a mixed sample of children aged 2–10 years with autism spectrum disorder (ASD), non-ASD developmental delays, and typically developing children across the full spectrum of cognitive ability. Consistent with published data on the original DAS and the MSEL, scores on the DAS-II and MSEL were highly correlated. However, curve estimation revealed large mean differences that varied as a function of the child’s cognitive ability level. We conclude that interchanging MSEL and DAS-II scores without regard to the discrepancy in scores may produce misleading results in both cross-sectional and longitudinal studies of children with and without ASD, and, thus, this practice should be implemented with caution.