1 of 1

Encoding/Target Stimulus Presentation

Recall/Test Stimulus Presentation

1000 ms

Target 2

1000 ms

Target 3

1000 ms

Target 4

1000 ms

Target 1

Response Condition

1000 ms

Test Stimulus 1

1000 ms

Test Stimulus 2

1000 ms

Test Stimulus 3

1000 ms

Test Stimulus 4

3500 ms

Response Period

3000 ms

Trial ISI

500 or 2000 ms

ISI

500 or 2000 ms

ISI

500 or 2000 ms

ISI

Introduction

  • Overall, results suggest that children with NF1 may experience deficits in both EF and academic skills and that problems in these two domains are likely to co-exist. Sustained attention is also related to academic difficulties across domains.
  • Due to this co-occurrence, interventions that target these skills should be well integrated. It also follows that interventions that target attention and EF may also result in improvements in academic achievement.
  • A better understanding of neurocognitive function in children with NF1 will promote the development of better screening and intervention protocols that will help minimize the extent of these difficulties in everyday functioning.

  • Consistent with the literature3, measures of full-scale IQ revealed low average intellectual functioning (M=84.70, SD=16.03).
  • Lower scores on measures of EF (e.g., inhibition of prepotent responses) was linked to lower math achievement scores (r=.73, p<.05).
  • More difficulties with sustained attention (e.g. higher omitted responses on attention task) were associated with lower performance on measures of both reading (r=-.60, p<.05) and math achievement (r=-.59, p<.05).
  • Parent report of more problems with EF was correlated with lower reading scores (r=-.68, p<.05).

Method

Discussion

Results continued

References

  • Neurofibromatosis is an autosomal dominant genetic disorder affecting approximately 100,000 people in the U.S. Neurofibromatosis Type 1 (NF1) is the most common type of neurofibromatosis, affecting 1 in 3,000-4,000 individuals.4
  • NF1 is most commonly associated with neurofibromas (tumors of peripheral nerves), café-au-lait spots (light brown spots on the skin), and optic gliomas, as well as several other musculoskeletal, skin, eye, and nervous system conditions.4
  • NF1 has been linked to neurocognitive deficits, including higher incidences of learning disabilities2 and ADHD1.
  • The current study examined relationships among attention, executive functions (EF), sustained attention, and academic achievement in children and adolescents diagnosed with NF1.
  • All participants were diagnosed with NF1 and were referred for neuropsychological evaluation as part of standard clinical care.
  • Relations between attention, EF, and academic achievement were examined using bivariate Pearson correlations with level of significance set at p<.05.

Demographic Characteristics

N=29

Age

M=9.28, SD=5.50

Range 2-21

Inhibition

Sustained Attention

Math

Reading

Parent Reported EF

Inhibition

1

Sustained Attention

-0.68*

1

Math

0.73*

-0.59*

1

Reading

0.461

-0.60*

0.735**

1

Parent Reported EF

-0.08

0.40

-0.54

-0.68*

1

Results

  1. Curatolo, P., Paloscia, C., D'Agati, E., Moavero, R., & Pasini, A. (2009). The neurobiology of attention deficit/hyperactivity disorder. European Journal of Paediatric Neurology, 13(4), 299-304.
  2. Hyman, S. L., Arthur, E., & North, K. N. (2006). Learning disabilities in children with neurofibromatosis type 1: subtypes, cognitive profile, and attention‐deficit‐hyperactivity disorder. Developmental Medicine & Child Neurology, 48(12), 973-977.
  3. Lehtonen, A., Howie, E., Trump, D., & Huson, S. M. (2013). Behaviour in children with neurofibromatosis type 1: Cognition, executive function, attention, emotion, and social competence. Developmental Medicine & Child Neurology, 55(2), 111-125.
  4. National Institutes of Health. (2011). Neurofibromatosis fact sheet. NIH Publication No. 11-2126.

* p<.05, **p<.01

Neurocognitive Functioning in Pediatric NF1: Executive Functions and Academic Achievement

Claire Friedhoff, MA1, Alison Oh, MS2, & Lea Ventura, PhD2

1 Roosevelt University, 2 University of Illinois at Chicago Medical Center

Domains Tested

Intellectual Abilities

WPPSI-IV; WISC-V; WAIS-IV (FSIQ)

Attention

CPT-3, K-CPT (Omissions)

Executive Functioning

DKEFS

(Color Word Interference: Inhibition)

Academic Achievement

WIAT-III (Word Reading Composite, Math Composite)

Parent Reported Executive Functioning

BRIEF-2 (Global Executive Composite)