Neurofibromatosis Type1 NF is an autosomal dominant disorder with mutation of chromosome 17' It occu

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Executive Functioning Predicts Social, Emotional,
and Adaptive Functioning in Children with
Neurofibromatosis Type 1Dawn L. Ilardi, Ph.D.,
Kathleen OToole, Ph.D. Thomas G. Burns,
Psy.D., ABPP-CN Childrens Healthcare of
Atlanta, Department of NeuropsychologyNational
Academy of Neuropsychology October 2008

• INTRODUCTION
• Neurofibromatosis Type-1 (NF) is an autosomal
  dominant disorder with mutation of chromosome 17.
  It occurs in 1/3500 and includes CNS
  abnormalities such as neurofibromas, optic
  gliomas (see Figure 1), macrocephaly, seizures,
  brain hyperintensities (see Figure 2), and an
  abnormal callosal area (see Figure 3). Brain
  hyperintensities are commonly seen in the basal
  ganglia, cerebellum, and white matter tracts and
  are posited to cause abnormal or delayed
  myelination. The neuropsychological effects of
  these neurological abnormalities are variable
  (Hyman et al., 2007).
• NF is also associated with intellectual (MR
  occurs in 3-6) and academic (30-69)
  disabilities. Hyman and colleagues (2006)
  reported three distinct cognitive profiles in
  children with NF Specific learning disability
  (average IQ, VIQ lt PIQ, academic lower) general
  learning difficulty (lower IQ and academic) and
  no learning disability (average IQ and academic)
  yet, all three groups had deficits in aspects of
  executive functioning. Extensive research
  supports deficits in attention/executive
  functioning in children with NF (e.g. Koth et
  al., 2000). Finally, children with NF have
  particular difficulty with nonverbal learning,
  including problems in the areas of social and
  emotional functioning (Barton and North, 2004
  Johnson et al., 1999). In summary, children
  with NF have variable intellectual and academic
  profiles, but deficits are relatively consistent
  for executive functioning, social, and emotional
  functioning.
• The purpose of the current study was to
  investigate whether executive functioning
  difficulties contribute to social, emotional, and
  behavioral challenges experienced by children
  with NF. Of particular interest is Rueda and
  colleagues (2005) model, which describes the
  behavioral and neurological contributions of
  executive functioning that contribute to adaptive
  self-regulation. In the context of this model,
  and given that children with NF tend to have
  executive functioning deficits as well as
  problems with social and emotional functioning,
  it was hypothesized that executive dysfunction
  would significantly predict externalizing and
  internalizing symptoms, as well as delays in
  daily adaptive functioning at home and school
  (i.e., social, conceptual, and practical).
• Figure 1. Optic Glioma
  Figure 2. Hyperintensities Figure 3.
  Corpus Callosum
• METHODS
• Twenty-three participants were selected from
  archival data based on a diagnosis of NF at a
  childrens hospital (Males 14 Ages 4-4 to
  18-7 years). A flexible neuropsychological
  battery was administered in an outpatient
  setting. To assess executive functioning,
  internalizing, and externalizing symptoms, data
  were included from parent and teacher forms of
  the Behavior Rating Inventory of Executive
  Functioning and the Behavioral Assessment System
  for Children. To assess adaptive functioning at
  home and school, the Adaptive Behavior Assessment
  System-2 Parent and teacher versions were
  included.

• RESULTS
• Pearson-r correlation analyses indicated
• Externalizing domain - Parent and teacher
  reports indicated that behavioral regulation
  (i.e., inhibition, emotional control) was related
  to aggression and conduct problems and working
  memory was related to conduct problems (p lt.01).
  
• Internalizing domain - Parent and teacher
  reports showed that behavioral regulation (i.e.,
  inhibition, shifting, emotional control) was
  related to anxiety and depression (plt.05).
• Adaptive functioning - Parent report indicated
  that only behavior regulation (i.e., inhibition,
  shifting, emotional control) predicted
  conceptual, social, and practical functioning
  whereas, teacher report indicated that both
  behavior regulation (i.e., inhibition, shifting)
  and metacognitive skills (i.e., working memory
  planning/organization, organization of materials,
  and self-monitoring) were related to conceptual,
  social, and practical functioning (plt.05).
• Multiple regression analyses revealed
• Externalizing domain - Behavioral regulation
  compared with metacognitive difficulties
  predicted externalizing problems (R2.69,
  F(4,21)4.54 (plt.01), with significant effects
  for inhibition (b.44, plt.01) and emotional
  control (b.30, plt.01).
• Internalizing domain - Behavioral regulation
  rather than metacognitive difficulties predicted
  internalizing problems (R2.55, F(4,21)3.52
  (plt.01), with significant effects for emotional
  control (b.35, plt.01) and shifting (b.21,
  plt.01).
• Adaptive functioning - Parent report indicated
  that behavioral regulation rather than
  metacognitive difficulties predicted overall
  adaptive functioning (R2.60, F(4,21)4.23
  (plt.01), with significant effects for inhibition
  (b.44, plt.01) and shifting (b.30, plt.05).
  Teacher report showed that metacognitive ability
  compared to behavioral regulation predicted
  adaptive functioning in the classroom (R2.43,
  F(4,21)3.53 (plt.05), with significant effects
  for working memory (b.20, p.01).
• RESULTS SUMMARY Executive functioning deficits
  contribute to maladaptive functioning in children
  with NF
• Disinhibition externalizing problems at home
  and school, delayed adaptive functioning at home
• Poor emotional control externalizing and
  internalizing problems at home and school
• Inflexibility/difficulty with transitions
  internalizing problems at home and school,
  delayed adaptive functioning at home
• Poor working memory delayed adaptive
  functioning at school
• CONCLUSION
• Analyses support the hypothesis that
  difficulties in executive functioning
  significantly relate to challenges in emotional
  functioning and adaptive functioning at home in
  school for children with NF. These findings also
  support the model proposed by Rueda and
  colleagues (2005). School and home interventions
  should focus on strategies to increase
  self-regulation to improve emotional and adaptive
  functioning. To improve adaptive functioning at
  school, interventions should target improving the
  childs working memory capabilities.