This study examined the degree to which parents are informed about syndrome-based behaviors in children with three genetic mental retardation syndromes: Down syndrome (n = 21), Prader-Willi syndrome (n = 25), and Williams syndrome (n = 21). Parents were informed regarding blatant behavioral features associated with their children's syndromes (especially concerning maladaptive behaviors) but were less informed about certain subtle syndrome-based cognitive processing outcomes. Compared to the parents of children with the other two syndromes, parents of children with Down syndrome were more informed about syndrome-related behaviors; in addition, greater numbers of individuals (e.g., parents, school psychologists, teachers, speech—language pathologists) brought information about children with Down syndrome into the classroom setting. Across all three groups, the supportive educational services received by the children were generally not adjusted to syndrome profiles.
Get full access to this article
View all access options for this article.
References
1.
Bellugi, U., Wang, P.P., & Jernigan, T.L. (1994). Williams syndrome: An unusual neuropsychological profile. In S. H. Broman & J. Grafman (Eds.), Atypical cognitive deficits in developmental disorders (pp. 23— 56). Hillsdale, NJ: Erlbaum.
2.
Dykens, E.M. (1995). Measuring behavioral phenotypes: Provocations from the “new genetics.” American Journal on Mental Retardation, 99, 522—532.
3.
Dykens, E.M. (in press). Anxiety, fears, and phobias in Williams syndrome. Journal of Developmental Neuropsychology.
4.
Dykens, E.M., & Cassidy, S.B. (1996). Prader-Willi syndrome: Genetic, behavioral, and treatment issues. Child and Adolescent Psychiatric Clinics of North America, 5, 913—927.
5.
Dykens, E.M., & Hodapp, R.M. (2001). Research in mental retardation: Toward an etiologic approach. Journal of Child Psychology and Psychiatry and Allied Disciplines, 42, 49—71.
6.
Dykens, E.M., Hodapp, R.M., & Finucane, B. (2000). Genetics and mental retardation syndromes: A new look at behavior and intervention. Baltimore: Brookes.
7.
Dykens, E.M., Hodapp, R.M., Walsh, K.K., & Nash, L. (1992). Profiles, correlates, and trajectories of intelligence in Prader-Willi syndrome. Journal of the American Academy of Child and Adolescent Psychiatry, 31, 1125—1130.
8.
Dykens, E.M., & Kasari, C. (1997). Maladaptive behavior in children with Prader-Willi syndrome, Down syndrome, and nonspecific mental retardation. American Journal on Mental Retardation, 102, 228—237.
9.
Dykens, E.M., Leckman, J.F., & Cassidy, S.B. (1996). Obsessions and compulsions in Prader-Willi syndrome . Journal of Child Psychology and Psychiatry, 37, 995—1002.
10.
Fowler, A. (1990). Language abilities in children with Down syndrome: Evidence for a specific syntactic delay. In D. Cicchetti & M. Beeghly (Eds.), Children with Down syndrome: A developmental perspective (pp. 302—328). Cambridge, UK: Cambridge University Press .
11.
Gresham, F., & Witt, J.C. (1997). Utility of intelligence tests for treatment planning, classification, and placement decisions: Recent empirical findings and future directions. School Psychology Quarterly, 12, 249—267.
12.
Hodapp, R.M. (1996). Down syndrome: Developmental, psychiatric, and management issues. Child and Adolescent Psychiatric Clinics of North America, 5, 881—894.
13.
Hodapp, R.M., Evans, D.W., & Gray, F.L. (1999). Intellectual development in children with Down syndrome. In J. Rondal, J. Perera, & L. Nadel (Eds.), Down syndrome: A review of current knowledge (pp. 124—132). London: Whurr.
14.
Hodapp, R.M., & Fidler, D.J. (1999). Special education and genetics: Connections for the 21st century. The Journal of Special Education, 22, 130—137.
15.
Hodapp, R.M., Leckman, J.F., Dykens, E.M., Sparrow, S.S., Zelinsky, D., & Ort, S.I. (1992). K-ABC profiles in children with Fragile X syndrome, Down syndrome, and non-specific mental retardation. American Journal on Mental Retardation, 97, 39—46.
Jarrold, C., Baddeley, A.D., & Hewes, A.K. (1999). Genetically dissociated components of working memory: Evidence from Down's and Williams syndrome. Neuropsychologia , 37, 637—651.
18.
Kingsley, J., & Levitz, M. (1994). Count us in. Orlando, FL: Harcourt, Brace & Company.
19.
Klein, B.P., & Mervis, C.B. (1999). Contrasting patterns of cognitive abilities of 9- and 10-year-olds with Williams syndrome or Down syndrome. Developmental Neuropsychology, 16, 177—196.
20.
Kumin, L. (1994). Intelligibility of speech in children with Down syndrome in natural settings: Parents' perspective. Perceptual and Motor Skills, 78, 307—313.
21.
Lenhoff, H.M. (1998). Insights into the musical potential of cognitively impaired people diagnosed with Williams syndrome. Music Therapy , 16, 33—36.
22.
Lloyd, J.W. (1984). How shall we individualize instruction—or should we?Remedial and Special Education, 5(1), 7—15.
23.
Mervis, C.B., Morris, C.A., Bertrand, J.M., & Robinson, B.F. (1999). Williams syndrome: Findings from an integrated program of research. In H. Tager-Flusberg (Ed.), Neurodevelopmental disorders: Contributions to a framework from the cognitive sciences (pp. 65—110). Cambridge, MA: MIT Press.
24.
Miller, J. (1999). Profiles of language development in children with Down syndrome. In J. F. Miller, M. Leddy, & L. A. Leavitt (Eds.), Improving the communication of people with Down syndrome (pp. 11—39). Baltimore: Brookes.
Pober, B.R., & Dykens, E.M. (1996). Williams syndrome: An overview of medical, cognitive, and behavioral features. Child and Adolescent Psychiatric Clinics of North America, 5, 929—943.
27.
Pueschel, S.R. (1990). Clinical aspects of Down syndrome from infancy to adulthood. American Journal of Medical Genetics, 7(Suppl. 7), 52—56.
28.
Pueschel, S.R., Gallagher, P.L., Zartler, A.S., & Pezzullo, J.C. (1987). Cognitive and learning profiles in children with Down syndrome. Research in Developmental Disabilities, 8, 21—37.
29.
Speece, D.L. (1990). Aptitude-treatment interactions: Bad rap or bad idea?The Journal of Special Education, 24, 139—149.
30.
Udwin, O., & Yule, W. (1991). A cognitive and behavioural phenotype in Williams syndrome. Journal of Clinical & Experimental Neuropsychology , 13, 232—244.
31.
Udwin, O., Yule, W., & Martin, N. (1987). Cognitive abilities and behavioural characteristics of children with idiopathic infantile hypercalcaemia. Journal of Child Psychology and Psychiatry and Allied Disciplines, 28, 297—309.
32.
Van Borsel, J., Curfs, L.M.G., & Fryns, J.P. (1997). Hyperacusis in Williams suyndrome: A sample survey . Genetic Counseling, 8, 121—126.
33.
Wang, P.P., & Bellugi, U. (1994). Evidence from two genetic syndromes for a dissociation between verbal and visual-spatial short-term memory. Journal of Clinical and Experimental Neuropsychology, 16, 317—322.
34.
Wilson, P.G., & Mazzocco, M.M.M. (1993). Awareness and knowledge of Fragile X syndrome among special educators. Mental Retardation , 31, 221—227.
35.
York, A., von Fraunhofer, N., Turk, J., & Sedgwick, P. (1999). Fragile X syndrome, Down's syndrome, and autism: Awareness and knowledge amongst special educators. Journal of Intellectual Disability Research, 43, 314—324.
