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53 Concurrent Cognitive Predictors of School Age Academic Functioning in Children with Neurofibromatosis Type 1
- Kristin M Lee, Ellen Olszewski, Brianna D Yund, Bonita P Klein-Tasman
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- Journal:
- Journal of the International Neuropsychological Society / Volume 29 / Issue s1 / November 2023
- Published online by Cambridge University Press:
- 21 December 2023, pp. 658-659
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Objective:
Children with neurofibromatosis type 1 (NF1) commonly have academic problems. While the neuropsychological profile of children with NF1 is variable, NF1 results in difficulties in a variety of cognitive domains including intellectual functioning, attention, executive functioning. Previous studies have suggested that cognitive functioning may relate to academic functioning in children with NF1; however, the evidence is limited and warrants further examination. Thus, this study will investigate the contribution of several cognitive domains (intellectual functioning, attention, working memory) on academic performance for school age children with NF1.
Participants and Methods:The association between cognitive functioning and academic performance was examined for school age children with NF1 (n = 40; ages 9-13). Intellectual functioning was assessed using the Differential Abilities Scales, Second Edition School-Age Version (DAS-II) General Conceptual Ability (GCA) score. Attention was examined using the DAS-II Recall of Digits-Forward (DF) subtest and Flanker Inhibitory Control and Attention Test (Flanker). Working memory was assessed using the DAS-II Recall of Digits-Backward (DB) subtest. Academic performance was measured using the Wechsler Individual Achievement Test, Third Edition (WIAT-III) Word Reading (WR), Pseudoword Decoding (PD), Reading Comprehension (RC), Numerical Operations (NO), and Math Problem Solving (MPS) subtests.
Results:WR was significantly associated with DAS-II GCA (rs(38) = .689, p < .001) and DF (rs(38) = .470, p = .002) in addition to Flanker (rs(34) = .364, p = .029), but not DAS-II DB (rs(38) = .292, p = .072). PD was significantly correlated with DAS-II GCA (rs(38) = .695, p < .001), DF (rs(38) = .394, p = .012), and DB (rs(38) = .474, p = .002), but not Flanker (rs(34) = .306, p = .070). RC was significantly associated with DAS-II GCA (rs(38) = .483, p = .002) and DF (rs(38) = .346, p = .029), but not DAS-II DB (rs(38) = .306, p = .055) and Flanker (rs(34) = .269, p = .112). NO was significantly correlated with DAS-II GCA fc(38) = .777, p < .001), DF (rs(38) = .555, p = < .001), and DB (rs(38) = .576, p < .001) as well as Flanker (rs(34) = .386, p = .020). MPS was significantly associated with DAS-II GCA (rs(38) = .685, p < .001), DF (rs(38) = .586, p < .001), and DB (rs(38) = .543, p < .001), in addition to Flanker (rs(34) = .420, p = .011). Significant associations had medium to large effect sizes, while non-significant correlations had small to medium effect sizes. Notably, most of the non-significant correlations had trend-level statistical significance.
Conclusions:Concurrent cognitive functioning (intellectual functioning, attention, working memory) was associated with reading-related and mathematics functioning in school age children with NF1. Notably, intellectual functioning had the strongest association with academic performance across all reading-related and mathematics tasks. Future studies should examine the association between academic performance and additional cognitive domains (e.g., language, visuospatial abilities) in children with NF1 across a wider age range to allow for examination of developmental patterns.
63 Comparison of Measures for Identification of Social Difficulties in Early Childhood for Children with Neurofibromatosis Type 1
- Danielle M Glad, Brianna D Yund, Kristin Lee, Christina L Casnar, Bonita P Klein-Tasman
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- Journal:
- Journal of the International Neuropsychological Society / Volume 29 / Issue s1 / November 2023
- Published online by Cambridge University Press:
- 21 December 2023, pp. 739-740
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Objective:
Social functioning patterns vary across measures in children with neurofibromatosis type 1 (NF1; Glad et al., 2021) with broad psychosocial screening measures having shown no impairment (Klein-Tasman et al., 2014; Martin et al., 2012; Sangster et al., 2011) while a more specific social functioning measure indicated poorer social skills (Barton & North, 2004; Huijbregts & de Sonneville, 2011; Loitfelder et al., 2015). The current aims were to characterize caregiver-reported social skills using three different measures and determine which measure appears to best capture social difficulties for young children with NF1.
Participants and Methods:Fifty children with NF1 (31 males; M=3.96, SD=1.05) and 20 unaffected siblings (11 males; M=4.34, SD=0.88) in early childhood (ages 3-6) were rated by a caregiver on one social functioning measure (the Social Skills scale on the Social Skills Rating System (SSRS)) and two broader functioning measures that include assessment of social functioning (the Social Skills scale on the Behavior Assessment System for Children-Second Edition (BASC-2), Social Interaction and Communication domain on the Scales of Independent Behavior-Revised (SIB-R)).
Results:For children with NF1, the SSRS mean standard score was significantly lower than the BASC-2 and SIB-R (f=-5.11, p<.001; f=-4.63, p<.001) while there was no significant difference between the BASC-2 and SIB-R. No significant differences emerged between measures for unaffected siblings. No significant group differences in mean standard score were found for the SSRS, BASC-2 or SIB-R. Fisher’s exact tests revealed the NF1 group had significantly more frequent difficulties than unaffected siblings on the BASC-2 (p=.017) but not on the SSRS or SIB-R. For both groups, Cochran’s Q tests determined a significant difference in the proportion of identified social difficulties across measures (NF1: X2(2)=16.33, p<.001; Siblings: X2(2)=9.25, p=.01). Follow up McNemar’s tests demonstrated significantly more difficulties reported on the SSRS compared to the BASC-2 for both groups (NF1: p<.001; Siblings: p=.016). Significantly more frequent difficulties were also reported on the SSRS compared to the SIB-R for the NF1 group (p=.002) but not for the unaffected siblings group. No difference in the frequency of difficulties was evident between the BASC-2 and SIB-R for either group.
Conclusions:Social skills difficulties appear to be best captured using the SSRS in young children, particularly for children with NF1 as this measure resulted in the lowest mean score and the greatest frequency of difficulties observed within the NF1 group. However, it is notable that group differences in comparison to unaffected siblings were not observed in mean score or frequency of difficulties, such that these young children with NF1 are not showing marked social challenges but rather, social difficulties may be mild when present at this age. Nevertheless, using a measure that specifically targets social functioning, rather than a measure where social functioning is merely a component of a broad measure, appears beneficial to capturing social difficulty. Using measures that best capture social difficulties will contribute to early identification and assessment of intervention effectiveness. Further work with additional age ranges and longitudinal trajectory is needed.
58 Parent Ratings of Internalizing and Externalizing Behaviors in Children with NF1 Across Childhood: A Longitudinal Investigation
- Sara K Pardej, Kristin M Lee, Bonita P Klein-Tasman
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- Journal:
- Journal of the International Neuropsychological Society / Volume 29 / Issue s1 / November 2023
- Published online by Cambridge University Press:
- 21 December 2023, pp. 663-664
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Objective:
The purpose of the present study is to characterize the trajectory of internalizing and externalizing behaviors in children with NF1 longitudinally from the early childhood period to the school age period on a broad psychosocial norm-referenced measure using linear mixed model growth curves.
Participants and Methods:Children with NF1 (n=28) were seen at least once between the ages of 3-8 years old and then again between the ages of 9-13 years old. Parents completed the Behavior Assessment System for Children (BASC) Second Edition; the version of the BASC administered depended on age (i.e., preschool form or child form). Linear mixed model growth curve analyses were used to examine the developmental trajectories of children with NF1 on the following scales, which were selected due to findings in the literature: Externalizing Problems, Internalizing Problems, Hyperactivity, Anxiety, Depression, Attention, and Executive Function. T-scores (M=50, SD=10) were used. Higher scores indicate more challenges.
Results:By using loess lines to qualitatively describe the patterns of ratings across time, it is evident that most scales (Externalizing Problems, Internalizing Problems, Hyperactivity, Attention Problems, Executive Function) demonstrated curvilinear trajectory patterns, with scores peaking in the 8-10-year-old range, then decreasing again. However, there was no statistically significant effect of age on any of the scales. Notably, trajectories largely included standard scores within the normative range (T-scores between 45-55).
Conclusions:Overall, the models also suggest that most children with NF1 are within the average range of functioning on all scales examined across the childhood period. Furthermore, with the exception of the Depression and Anxiety scales, ratings tend to peak around the 8-10-year period, and then decrease into early adolescence. Thus, when working with patients with NF1, it may be the case that clinicians note relative increases in challenges across these domains in late childhood, though these challenges may decrease over time during this age range. Linear growth curve modeling identified that the developmental trajectories of internalizing and externalizing behaviors of children with NF1, as rated by parents, remain stable across the childhood period. Importantly, low power may have contributed to the lack of observed age effects. Longitudinal research would be beneficial to capture patterns that may emerge in adolescence or adulthood.
Cognitive and Psychosocial Phenotype of Young Children with Neurofibromatosis-1
- Bonita P. Klein-Tasman, Kelly M. Janke, Wen Luo, Christy L. Casnar, Scott J. Hunter, James Tonsgard, Pamela Trapane, Faye van der Fluit, Lorri A. Kais
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- Journal:
- Journal of the International Neuropsychological Society / Volume 20 / Issue 1 / January 2014
- Published online by Cambridge University Press:
- 15 November 2013, pp. 88-98
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Children with neurofibromatosis-1 (NF1), a neurodevelopmental disorder resulting from a mutation of the NF1 gene (17q11.2), often have difficulties with learning and attention, but there is little research in the early childhood years. In this study, the cognitive and psychosocial functioning of 40 young children with NF1 (ages 3 through 6) was examined and compared both to normative data and to a contrast group comprised of unaffected siblings and community members matched for age and socio-economic status (n = 37). Children with NF1 showed significantly weaker cognitive abilities across all domains and for the vast majority of subtests. Consistent with research in older children, a variety of patterns of intra-individual strength and weakness were present for young children with NF1. Few significant group differences in psychosocial functioning were observed, but the children with NF1 showed significantly greater functional communication problems than did the unaffected group. Overall, the results indicate that in participant groups matched for age and socioeconomic status, cognitive vulnerabilities are evident for close to half of young children with NF1, with some relations to psychosocial functioning, particularly functional communication, attention problems and social skills. (JINS, 2014, 1, 1–11)
11 - Genetic syndromes associated with intellectual disability
- Edited by Scott J. Hunter, University of Chicago, Jacobus Donders, Mary Free Bed Rehabilitation Hospital, Grand Rapids
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- Pediatric Neuropsychological Intervention
- Published online:
- 13 August 2009
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- 03 May 2007, pp 193-223
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Summary
A central role of pediatric neuropsychologists is to apply an understanding of neurocognitive disorders to the assessment of a child's pattern of strengths and weaknesses, and to point families in the direction of appropriate interventions (Baron, 2003; Bernstein, 2000). In recent years, with advances in molecular and clinical genetics, there has been a surge in research examining the phenotypes associated with a broad range of genetically based neurodevelopmental disorders, in the search for a better understanding of genotype–phenotype relations (Denckla, 2000; Fisch, 2000). This knowledge is invaluable to neuropsychologists who can inform their assessments based on these investigations. In addition, knowledge about etiology has the potential to inform intervention suggestions, including recommendations to support transition to adulthood, once a body of knowledge about the impact of such interventions exists.
In this chapter, we discuss the importance of consideration of etiology in intervention planning for children with mental retardation syndromes. First, the potential strengths of an etiological approach, in contrast to one that focuses primarily on the level of functioning of the child, are described. Secondly, an outline of the assessment targets that are typically addressed with children with mental retardation is presented. Thirdly, the cognitive and behavioral phenotypes characteristic of a number of genetic syndromes with associated intellectual disability are presented, with a focus on available literature about appropriate interventions targeted to particular genetic disorders.