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Behavioral Phenotypes in Children with Down Syndrome, Prader-Willi Syndrome, or Angelman Syndrome

Abstract

This study investigated the concept of behavioral phenotypes by comparing behavior ratings of 91 children with Down syndrome, 28 children with Prader-Willi syndrome, 68 children with Angelman syndrome, and 24 children with nonspecific mental retardation. Results supported distinctive behavioral profiles for the syndrome groups. The Down syndrome group showed good social skills and low ratings of problem behaviors including hyperactivity. Children with Prader-Willi syndrome exhibited a pattern of externalizing, obsessive–compulsive, anxious, and overly sensitive behaviors. A profile of a happy disposition, hand clapping, mouthing behaviors, and severe levels of hyperactivity and attention problems emerged for children with Angelman syndrome. Implications for clinical practice and future research were discussed.

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REFERENCES

  • Achenbach, T. M., and Edelbrock, C. (1983). Manual for the Child Behavior Checklist, University of Vermont Department of Psychiatry, Burlington, VT.

  • Aman, M. G., Singh, N. N., Stewart, A. W., and Field, C. J. (1985). The Aberrant Behavior Checklist: A behavior rating scale for the assessment of treatment effects. Am. J. Ment. Deficiency89: 485–491.

    Google Scholar 

  • Aman, M. G., Tasse, M. J., Rojahn, J., and Hammer, D. (1996). The Nisonger CBRF: A child behavior rating form for children with developmental disabilities. Res. Dev. Disabil. 17: 41–57.

    Google Scholar 

  • Angelman, H. (1965). Puppet children: A report on three cases. Dev. Med. Child Neurol.7: 681–688.

    Google Scholar 

  • Clarke, D. J., and Marston, G. (2000). Problem behaviors associated with 15q-Angelman syndrome. Am. J. Ment. Retard. 105: 25–31.

    Google Scholar 

  • Cohen, M., and Hynd, G. W. (1986). The Conners Teacher Rating Scale: A different factor structure with special education children. Psychol. Schools 23: 13–23.

    Google Scholar 

  • Cuskelly, M., and Dadds, M. (1992). Behavioural problems in children with Down's syndrome and their siblings. J. Child Psychol. Psychiatry 33: 749–761.

    Google Scholar 

  • Dimitropoulos, A., Feurer, I.D., Butler, M.G., and Thompson, T. (2001). Emergence of compulsive behavior and tantrums in children with Prader-Willi syndrome. Am. J. Ment. Retard. 106: 39–51.

    Google Scholar 

  • Dykens, E.M. (1995). Measuring behavioral phenotypes: Provocations from the “new genetics.” Am. J. Ment. Retard. 99: 522–532.

    Google Scholar 

  • Dykens, E.M. (1996).DNAmeets DSM: The growing importance of genetic syndromes in dual diagnosis. Ment. Retard. 34: 125–127.

    Google Scholar 

  • Dykens, E. M., and Cassidy, S. B. (1996). Prader-Willi syndrome: Genetic, behavioral and treatment issues. Child Adolesc. Psychiatr. Clin. North Am. 5: 913–927.

    Google Scholar 

  • Dykens, E. M., and Kasari, C. (1997). Maladaptive behavior in children with Prader-Willi syndrome, Down syndrome, and mixed mental retardation. Am. J. Ment. Retard. 102:228–237.

    Google Scholar 

  • Dykens, E. M., Leckman, J. F., and Cassidy, S. B. (1996). Obsessions and compulsions in Prader-Willi syndrome. J Child Psychol. Psychiatry 37: 995–1002.

    Google Scholar 

  • Edelbrock, C. S. (1985). Child Behavior Rating Form. Psychopharmacol. Bull. 21: 835–837.

    Google Scholar 

  • Einfeld, S. L., and Aman, M. G. (1995). Issues in the taxonomy of psychopathology in children and adolescents with mental retardation. J. Autism Dev. Disord. 25: 143–167.

    Google Scholar 

  • Fidler, D. J., and Hodapp, R. M. (1998). Importance of typologies for science and service in mental retardation. Ment. Retard. 36: 489–495.

    Google Scholar 

  • Harris, J. C. (1995). Developmental Neuropsychiatry: Vol. II, Oxford University Press, Oxford.

    Google Scholar 

  • Hodapp, R. M. (1996). Down syndrome: Developmental, psychiatric, and management issues. Child Adolesc. Psychiatr. Clin. North Am. 5: 881–894.

    Google Scholar 

  • Hodapp, R. M., and Dykens, E.M. (2001). Strengthening behavioral research on genetic mental retardation syndromes. Am. J. Ment. Retard. 106: 4–15.

    Google Scholar 

  • Holm, V. A., Cassidy, S. B., Butler, M. G., Hanclott, J. M., Greenswag, L. R., Whitman, L., and Greenberg, F. (1993). Prader-Willi syndrome: Consensus diagnostic criteria. Pediatrics 91: 398–402.

    Google Scholar 

  • Magenis, R. E., Toth-Fejel, S., Allen, L. J., Black, M., Brown, M. G., Budden, S., Cohen, R., Friedman, J. M., Kalousek, D., Zonana, J., Lace, D., LaFranchi, S., Lahr, M., MacFarlane, J., and Williams, C.P. S. (1990). Comparison of the 15q deletions in Prader-Willi and Angelman syndromes: Specific regions, extent of deletions, parental origin and clinical consequences. Am J. Med. Gen. 35: 333–349.

    Google Scholar 

  • Matson, J. L., Epstein, M. H., and Cullinan, D. (1984). A factor analytic study of the Quay-Peterson scale with mentally retarded adolescents. Educ. Training Ment. Retard. 19: 150–154.

    Google Scholar 

  • Meyers, B. A., and Pueschel, S. M. (1991). Psychiatric disorders in persons withDownsyndrome. J. Nerv. Ment. Dis. 179: 609–613.

    Google Scholar 

  • Nicholls, R. D. (1993). Genomic imprinting and uniparental disomy in Angelman and Prader-Willi syndromes: A review. Am. J. Med. Gen. 46: 16–25.

    Google Scholar 

  • Peterson, B. S. (1995). Neuroimaging in child and adolescent neuropsychiatric disorders. J. Am. Acad. Child Adolesc. Psychiatry 34: 1560–1576.

    Google Scholar 

  • Pueschel, S. M., and Myers, B. A. (1994). Environmental and temperament assessments of children with Down's syndrome. J. Intellect. Disabil. Res. 38: 195–202.

    Google Scholar 

  • Pulsifer, M. B. (1996). The neuropsychology of mental retardation. J. Int. Neuropsychol. Soc. 2: 159–176.

    Google Scholar 

  • Roof, E., Stone, W., MacLean, W., Feurer, I. D., Thompson, T., and Butler, M. G. (2000). Intellectual characteristics of Prader-Willi syndrome: Comparison of genetic subtypes. J. Intellect. Disabil. Res. 44: 25–30.

    Google Scholar 

  • Summers, J. A., and Feldman, M. A. (1999). Distinctive pattern of behavioral functioning in Angelman syndrome. Am. J. Ment. Retard. 104: 376–384.

    Google Scholar 

  • Swaab, D. F. (1997). Prader-Willi syndrome and the hypothalamus. Acta Paediatr. Suppl. 423: 50–54.

    Google Scholar 

  • Tasse, M. J., Aman, M. G., Hammer, D., and Rojahn, J. (1996). The Nisonger child behavior rating form: Age and sex effects and norms.Res. Dev. Disabil. 17: 59–75.

    Google Scholar 

  • Turk, J., and Sales, J. (1996). Behavioural phenotypes and their relevance to child mental health professionals. Child Psychol. Psychiatry Rev. 1: 4–11. Neurogenetic Syndromes and Behavioral Phenotypes 321

    Google Scholar 

  • Warren, A. C., Holroyd, S., and Folstein, M. F. (1989). Major depression in Down's syndrome. Br. J. Psychiatry 155: 202–205.

    Google Scholar 

  • Williams, C. A., Angelman, H., Clayton-Smith, J., Driscoll, D. J., Hendrickson, J. E., Knoll, J. H. M., Magenis, E., Schinzel, A., Wagstaff, J., Whidden, E. M., and Zori, R. T. (1995). Angelman syndrome: Consensus for diagnostic criteria. Am. J. Med. Genet. 56: 237–238.

    Google Scholar 

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Correspondence to Nicolay Chertkoff Walz.

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Walz, N.C., Benson, B.A. Behavioral Phenotypes in Children with Down Syndrome, Prader-Willi Syndrome, or Angelman Syndrome. Journal of Developmental and Physical Disabilities 14, 307–321 (2002). https://doi.org/10.1023/A:1020326701399

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  • DOI: https://doi.org/10.1023/A:1020326701399

  • Down
  • Prader-Willi
  • Angelman
  • syndrome
  • behavior