Pragmatic Profile Relative to Cognition in Children with Sotos Syndrome' Poster Board 111:

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Pragmatic Profile Relative to Cognition in
Children with Sotos Syndrome.Poster Board 111
1024

• Crystal Ahlborg, Graduate Student, University of
  Nebraska at Omaha
• Melissa Wong, Graduate Student, University of
  Nebraska at Omaha
• Mary Friehe, Ph.D., CCC-S, University of Nebraska
  at Omaha
• Mitzi Ritzman, Ph.D., CCC-S, University of
  Nebraska at Omaha
• Laura Ball, Ph.D., CCC-S, University of Nebraska
  Medical Center
• Brad Schaefer, M.D., Associate Director of
  Munroe-Meyer Institute
• at the University of Nebraska Medical Center

2
Literature Review

• What is Sotos Syndrome?
• Low incidence disorder only 350 cases reported
  worldwide (Adhami Cancio-Babu, 2003).
• Described as cerebral gigantism (Finegan, Cole,
  Kingwell, Smith, Smith, Sitarenios 1994) with
  increased height and weight that tends to
  normalize with age (Schaefer, Bodensteiner,
  Buehler, Lin, Cole, 1997)
• Reports of accompanying cognitive, motor,
  language, and social challenges
• pervasive verbal cognitive challenges and
  concomitant behavior / social skill problems
  (Anderson Buehler, 2000).
• expressive language, speech production, voice and
  fluency problems, but average social-behavioral
  pragmatic interactions (Ball, Sullivan, Dulany,
  Stading, Schaefer, 2005)

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Literature Review

• At Issue
• Link between cognition and language in Sotos
  Syndrome is unclear. The debate is whether or
  not the language characteristics are Sotos
  specific or a result of cognitive level (Finegan
  et al., 1994).
• There is some support to indicate that the
  pragmatic / problem behaviors in Sotos do appear
  to be syndrome specific and not a function of
  cognition (Sarimski, 2003).

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Purpose

• Research Question 1 What is the relation between
  cognition and pragmatic interaction skills in
  children with Sotos Syndrome?
• Research Question 2 What patterns of pragmatic
  and language interactions are identifiable in a
  Sotos Syndrome population?

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Methods

• Participants
• Drawn from existing database (Ball et al., 2005)
• n9 children with Sotos Syndrome
• Ages 1.4 years to 12.3 years
• Gender 5 girls and 4 boys

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Instruments Research Question 1

• Cognitive Correlate
• Peabody Picture Vocabulary Test III (PPVT)
• Pragmatic Interaction Measures
• Social Skills Rating System (SSRS) (Gresham
  Elliot, 1990)
• Parent rating with a standardized 3-point Likert
  rating scale (0 never/not important / 2 very
  often/critical)
• Utilized only Social Skills and Problem Behavior
  Scales

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Instruments Research Question 1

• Child Social Interaction Scale (CSIS) (Ball et
  al., 2005)
• An informal 1-4 Likert rating scale completed by
  parent to describe the childs communication
  interactions (1 never / 4 very much).
• Consists of 5 subscales with 10 items each
• Communication Anxiety Your child avoids
  
• speaking in a group/classroom if possible.
• Communication Adaptability Your child
  enjoys
• socializing in various groups of children.
• Communication Competence Your child is
  a
• good listener.
• Conversational Appropriateness Your
  child says
• embarrassing things.
• Willingness to communicate Your child
  prefers to
• talk than to listen.

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Instruments Research Question 1

• Additional Language Measures
• Language sample with MLU, TTR using SALT (1991)
• Clinical Evaluation of Linguistic Fundamentals
  3 Screening Test
• Preschool Language Scale 3

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Results

• RESEARCH QUESTION 1
• No significant correlations (plt.05) between
  pragmatics and cognition when using the PPVT-III
  as cognitive indicator.
• (PPVT-III Mean 81.4 Range 55-105)
• Correlation Coefficients Score
• PPVT-III SSRS Range
• CSIS Adaptability p.831 p.907 53-90 (RS)
• CSIS Willingness p.84 p.273 45-90 (RS)
• SSRS Problem p..966 90-126 (SS)
• RSRaw Score SSStandard Score
• Informal comparison of three, cognitive level
  groupings did not result in trends for pragmatic
  skills.
• High Group PPVT score of 85-115 (n3)
• Low Average Group PPVT of 71-84 score (n4)
• Low Group PPVT score of 70 or below (n2)

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Results

• RESEARCH QUESTION 2
• Significant correlations were found between
• Pragmatic CSIS Communication Adaptability and
  Willingness subscales (Rho.69, p.01)
• Pragmatic CSIS Communication Adaptability and
  SSRS Score for Problem Behaviors (Rho-.684,
  p.04)
• No other significant correlations were found for
  any of the language variables.

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Correlations Graph
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Conclusions

• Research Question 1
• There were no statistically significant
  relationships between pragmatic interaction
  skills and cognition.
• Sotos Syndrome may be better defined within a
  spectrum rather than in the confines of one,
  homogeneous cluster of characteristics. This
  could explain the discrepancies identified in
  genetic research.
• There were no apparent syndrome-specific language
  or behavior characteristics.

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Conclusions

• Research Question 2
• Children who are rated as willing to communicate
  by parents also are rated as more willing to
  adapt in social situations.
• Children who are rated as less willing to adapt
  in social situations by parents using the CSIS
  also are rated as having more social behavior
  problems.

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Limitations

• Small sample size
• Problems comparing across instruments due to wide
  range of ages
• IQ was established utilizing only the PPVT
  limited by nature of extant data base

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Future Research

• Expand the sample size with equal representation
  in various cognitive categories OR within
  specific age groups so that equivalent
  instrumentation is available for comparisons
• Incorporate more valid and reliable indicators of
  cognition
• Continue to explore the efficacy of the CSIS or
  include an instrument that evaluates pragmatic
  language

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References

• Adhami, E., Cancio-Babu, C. (2003). Anesthesia
  in a child with Sotos syndrome. Pediatric
  Anesthesia, 13, 835-840.
• Anderson R., Buehler, B. (2000). What is Sotos
  syndrome? Sotos syndrome a handbook for
  families. Retrieved 1/29/07 from
  http//www.well.com/user/sssa/whatisit.htm
• Ball, L., Sullivan, M., Dulany, S., Stading, K.,
  Schaefer, B. (2005). Speech-language
  characteristics of children with Sotos syndrome.
  American Journal of Medical Genetics, 136A,
  363-367.
• Bell, N., Lassiter, K., Matthews, D.,
  Hutchinson, M. (2001). Comparison of the Peabody
  Picture Vocabulary Test Third Edition and
  Wechsler Adult Intelligence Scale Third Edition
  with university students. Journal of Clinical
  Psychology, 57(3), 417 422.
• Burgoon, J.K. (1976). Thw unwillingness-to-communi
  cate scale development and validation.
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• Dunn, L.M., Dunn, L.M. (1997). Peabody Picture
  Vocabulary-Third Edition (PPVT-III). Circle
  Pines, MN American Guidance Service.
• Duran, R.L. (1991). Communication adaptability a
  review of conceptualization and measurement.
  Communication Quarterly, 40, 253-268.
• Finegan J., Cole T., Kingwell E., Smith M., Smith
  M., Sitarenios G. (1994). Language and behavior
  in children with Sotos syndrome. Journal of
  Academic Child Adolescent Psychiatry, 33,
  1307-1315.
• Horikoshi, H., Kato, Z., Masuno, M., Asano, T.,
  Nagese, T., Yamagishi, Y.,Kozawa, R., Arai, T.
  (2006). Neuroradiologic findings in Sotos
  syndrome. Journal of Child Neurology, 21(7),
  614-618.
• McCroskey, J.C. (1992). Reliability and validity
  of the willingmess to communicate scale.
  Communication Quarterly, 40, 16-25.
• Sarimski, K. (2003). Behavioral and emotional
  characteristics in children with Sotos syndrome
  and learning disabilities. Developmental
  Medicine and Child Neurology, 45, 172-178.
• Shaefer, G.B., Bodensteiner, J.B., Buehler, B.A.,
  Lin, A., Cole, T.R. (1997). The neuroimaging
  findings in Sotos syndrome. American Journal of
  Medical Genetics, 68, 462-465.
• Weschler, D. (1955). Weschler adult intelligence
  scale. New York Psychological Corporation.