Chapter Seven - Social-perceptual and social-cognitive skills in young children with williams syndrome: Evidence for discontinuity

International Review of Research in Developmental Disabilities

Volumn 40, Issue 1, 2011, Pages 181-210

  Hepburn, Susan L ^a   Fidlery, Deborah J ^b   Hahny, Laura ^b   Philofsky, Amy ^a  

^a UNIVERSITY OF COLORADO   (United States)

^b Veterinary Dentistry and Oral Surgery   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

EID: 84866544309     PISSN: 22116095     EISSN: None     Source Type: Book Series    
DOI: 10.1016/B978-0-12-374478-4.00007-1     Document Type: Article

References (103)

1. Alegria, J., & Noirot, E. (1978). Neonate orientation behaviour towards human voice. International Journal of Behavioral Development, 1(4), 291-312. doi: 10.1016/S0163-6383(78)80040-X.
2. Arnold, R., Yule, W., & Martin, N. (1985). The psychological characteristics of infantile hypercalcaemia: A preliminary investigation. Developmental Medicine & Child Neurology, 27(1), 49-59.
3. Bakeman, R., & Adamson, L. (1984). Coordinating attention to people and objects in mother-infant and peer-infant interactions. ChildDevelopment, 55, 1278-1289. doi: 10.1111/1467-8624.ep7302943.
4. Baron-Cohen, S. (1989). Joint attention deficits in autism: Towards a cognitive analysis. Development and Psychopathology, 1, 185-190. doi: 10.1017/S0954579400000377.
5. Bartsch, K., & Estes, D. (1996). Individual differences in children's developing theory of mind and implications for metacognition. Learning and Individual Differences, 8(4), 281-304.
6. Bates, E. (1976). Language and context:The acquisition of pragmatics. New York: Academic Press.
7. Bellugi, U., Adolphs, R., Cassady, C., & Chiles, M. (1999). Towards the neural basis for hypersociability in a genetic syndrome. NeuroReport, 10, 1653-1657.
8. Bellugi, U., Lichtenberger, L., Jones, W., Lai, Z., & St George, M. (2000). I. The neurocognitive profile of Williams syndrome: A complex pattern of strengths and weaknesses. Journal of Cognitive Neuroscience, 12(1), 7-29. doi: 10.1162/089892900561959.
9. Bellugi, U., Wang, P. P., & Jernigan, T. L. (1994). Williams syndrome: An unusual neuropsychological profile. In S. H. Broman., J. Grafman., S. H. Broman, and J. Grafman, (Eds.), Atypical cognitive deficits in developmental disorders: Implications for brain function (pp. 23-56). Hillsdale, NJ England: Lawrence Erlbaum Associates, Inc. R.
10. Bertrand, J., Mervis, C. B., & Eisenberg, J. D. (1997). Drawing by children with Williams syndrome: A developmental perspective. Developmental Neuropsychology, 13(1), 41-67. doi: 10.1080/87565649709540667.
11. Berument, S., Rutter, M., Lord, C., Pickles, A., & Bailey, A. (1999). Autism screening questionnaire: Diagnostic validity. British Journal of Psychiatry, 175, 444-451. doi: 10.1192/bjp.175.5.444.
12. Beuren, A. J., Apitz, J., & Harmjanz, D. (1962). Supravalvular aortic stenosis in association with mental retardation and a certain facial appearance. Circulation, 26, 1235-1240.
13. Celani, G. (2004). Comorbidity between autistic syndrome and biological pathologies: Which implications for the understanding of the etiology? Journal of Developmental and Physical Disabilities, 16(2), 215-228. doi: 10.1023/B:JODD.0000026617.62533.44.
14. Davies, M., Udwin, O., & Howlin, P. (1998). Adults with Williams syndrome: Preliminary study of social, emotional and behavioural difficulties. British Journal of Psychiatry, 172, 273-276. doi: 10.1192/bjp.172.3.273.
15. DeCasper, A. J., & Fifer, W. P. (1980). Of human bonding: Newborns prefer their mothers' voices. Science, 208(4448), 1174-1176.
16. Dodd, H. F., Schniering, C. A., & Porter, M. A. (2009). Beyond behaviour: Is social anxiety low in Williams syndrome? Journal of Autism and Developmental Disorders, 39 (12), 1673-1681. doi: 10.1007/s10803-009-0806-4.
17. Don, A. J., Schellenberg, E., & Rourke, B. P. (1999). Music and language skills of children with Williams syndrome. Child Neuropsychology, 5(3), 154-170. doi: 10.1076/chin.5.3.154.7337.
18. Dykens, E. M., & Rosner, B. A. (1999). Refining behavioral phenotypes: Personalitymotivation in Williams and Prader-Willi syndromes. American Journal on Mental Retardation, 104, 158-169. doi: 10.1352/0895-8017(1999)104<0158: RBPPIW>2.0.CO;2.
19. Einfeld, S. L., Tonge, B. J., & Florio, T. (1997). Behavioral and emotional disturbance in individuals with Williams syndrome. American Journal on Mental Retardation, 102, 45-53. doi: 10.1352/0895-8017(1997)102<0045:BAEDII>2.0.CO;2.
20. Einfeld, S. L., Tonge, B. J., & Rees, V. W. (2001). Longitudinal course of behavioral and emotional problems in Williams syndrome. American Journal of Mental Retardation, 106, 73-81. doi: 10.1352/0895-8017(2001)106<0073:LCOBAE>2.0.CO;2.
21. Ewart, A., Morris, C., Atkinson, D., Jin, W., Sternes, K., Spallone, P., et al., (1993). Hemizygosity at the elastin locus in a developmental disorder, Williams syndrome. Nature Genetics, 5(1), 11-16.
22. Fidler, D. J., Hepburn, S. L., Most, D. E., Philofsky, A., & Rogers, S. J. (2007). Emotional responsivity in young children with Williams syndrome. American Journal of Mental Retardation, 112(3), 194-206. doi: 10.1352/0895-8017(2007)112 [194:ERIYCW]2.0.CO;2.
23. Francke, U. (1999). Williams-Beuren syndrome: Genes and mechanisms. Human Molecular Genetics, 8(10), 1947-1954.
24. Garfield, J. L., Peterson, C. C., & Perry, T. (2001). Social cognition, language acquisition, and development of theory of mind. Mind and Language, 6, 494-541.
25. Gosch, E. A., & Pankau, R. (1997). Personality characteristics and behaviour problems in individuals of different ages with Williams syndrome. Developmental Medicine and Child Neurology, 39, 527-533.
26. Grant, J., Karmiloff-Smith, A., Berthoud, I., & Christophe, A. (1996). Is the language of people with Williams syndrome mere mimicry? Phonological verbal short-term memory in a foreign language. Cahiers de Psychologie Cognitive, 15, 615-628.
27. Grant, J., Karmiloff-Smith, A., Gathercole, S. A., Paterson, S., Howlin, P., Davies, M., et al., (1997). Phonological short-term memory and its relationship to language in Williams syndrome. Cognitive Neuropsychiatry, 2, 81-99. doi: 10.1080/135468097396342.
28. Greenspan, S. I., & Wieder, S. (1999). A functional developmental approach to autism spectrum disorders. Journal of Association of Severe Handicaps, 24, 147-161.
29. Hobson, R. P. (1993). Autism and the development of mind. Hillsdale, NJ: Lawrence Erlbaum.
30. Huang, L., Sadler, L., O'Riordan, M., & Robin, N. H. (2002). Delay in diagnosis of Williams syndrome. Clinical Pediatrics, 41(4), 257-261. doi: 10.1177/000992280204100410.
31. Jarrold, C., Baddeley, A., & Hewes, A. (1998). Verbal and nonverbal abilities in the Williams syndrome phenotype: Evidence for diverging developmental trajectories. Journal of Child Psychology and Psychiatry, 39, 511-523.
32. Jarrold, C., Cowen, N., Hewes, A. K., & Riby, D. M. (2004). Speech timing and verbal short-term memory: Evidence for contrasting deficits in Down syndrome and Williams syndrome. Journal ofMemory and Language, 51(3), 365-380. doi: 10.1016/j.jml.2004.06.007.
33. Järvinen-Pasley, A., Adolphs, R., Yam, A., Hill, K. J., Grichanik, M., Reilly, J., et al., (2010). Affiliative behavior in Williams syndrome: Social perception and real-life social behavior. Neuropsychologia, 48(7), 2110-2119. doi: 10.1016/j.neuropsychologia.2010.03.032.
34. Järvinen-Pasley, A., Bellugi, U., Reilly, J., Mills, D. L., Galaburda, A., Reiss, A. L., et al., (2008). Defining the social phenotype in Williams syndrome: A model for linking gene, the brain, and behavior. Development and psychopathology, 20(1), 1-35. doi: 10.1017/S0954579408000011.
35. Jones, W., Bellugi, U., Lai, Z., Chiles, M., Reilly, J., Lincoln, A., et al., (2000). II. Hypersociability in Williams syndrome. Journal of Cognitive Neuroscience, 12(1), 30-46. doi: 10.1162/089892900561968.
36. Karmiloff-Smith, A. (1998). Development itself is the key to understanding developmental disorders.Trends in Cognitive Sciences, 2, 389-398. doi: 10.1016/S1364-6613(98)01230-3.
37. Karmiloff-Smith, A., Grant, J., Berthoud, I., Davies, M., Howlin, P., & Udwin, O. (1997). Language and Williams syndrome: How intact is "intact"? Child Development, 68, 246-262. doi: 10.1111/1467-8624.ep9706130497.
38. Karmiloff-Smith, A., Klima, E., Bellugi, U., Grant, J., & Baron-Cohen, S. (1995). Is there a social module? Language, face processing, and theory of mind in individuals with Williams syndrome. Journal of Cognitive Neuroscience, 7, 196-208. doi: 10.1162/jocn.1995.7.2.196.
39. Kasari, C. Jahromi, L., Freeman, S., & Paparella, T. (2006). Predictors of language growth in young children with autism. Paper Presented at the Gatlinburg Conference on Research and Theory in Intellectual and Developmental Disabilities, San Diego, CA.
40. Kasari, C., Sigman, M., Mundy, P., & Yirmiya, N. (1990). Affective sharing in the context of joint attention interactions of normal, autistic, and mentally retarded children. Journal of Autism and Developmental Disorders, 20(1), 87-100. doi: 10.1007/BF02206859.
41. Klein, B. P., & Mervis, C. B. (2000). Contrasting patterns of cognitive abilities of 9- and 10-year-olds with Williams syndrome or Down syndrome. Developmental Neuropsychology, 16(2), 177-196. doi: 10.1207/S15326942DN1602_3.
42. Klein-Tasman, B. P., Mervis, C. B., Lord, C., & Phillips, K. D. (2007). Socio-communicative deficits in young children with Williams syndrome: performance on the autism diagnostic observation schedule. Child Neuropsychology, 13(5), 444-467. doi: 10.1080/09297040601033680.
43. Klein-Tasman, B. P., Phillips, K. D., Lord, C., Mervis, C. B., & Gallo, F. J. (2009). Overlap with the autism spectrum in young children with Williams syndrome. Journal of Developmental and Behavioral Pediatrics, 30(4), 289-299. doi: 10.1097/DBP.0b013e3181ad1f9a.
44. Klinnert, M. D., Campos, J. J., Sorce, J. F., Emde, R. N., & Svejda, M. (1983). Emotions as behavior regulators: Social referencing in infancy. In R. Plutchik, and H. Kellerman, (Eds.), Emotion, theory, research, and experience New York: Academic Press.
45. Knowland, V., & Thomas, M. S. C. (2011). Developmental trajectories in genetic disorders. International Review of Research in Developmental Disabilities., 40.
46. Laing, E., Butterwoth, G., Ansari, D., Gsoedl, M., Longhi, E., Panagiotaki, G., et al., (2002). Atypical development of language and social communication in toddlers with Williams syndrome. Developmental Science, 5, 233-246.
47. Lincoln, A., Lai, Z., & Jones, W. (2002). Shifting attention and joint attention dissociation in Williams syndrome: Implications for the cerebellum and social deficits in autism. Neurocase, 8, 226-232. doi: 10.1093/neucas/8.3.226.
48. Lord, C., & Rutter, M. (1994). Autism Diagnostic Interview-Revised: A revised of a diagnostic interview for caregivers of individuals with possible pervasive developmental disorders. Journal of Autism and Developmental Disorders, 24(5), 659-685.
49. Lord, C., Rutter, M., DiLavore, P., & Risi, S. (1999). Autism diagnostic observation schedule-WPS edition. Los Angeles, CA: Western Psychological Services.
50. Maurer, D., & Salapatek, P. (1976). Developmental changes in the scanning of faces by young infants. Child Development, 47(2), 523-527. doi: 10.1111/1467-8624.ep12190048.
51. Meltzoff, A. (1988). Infant imitation after a 1-week long delay: Long-term memory for novel acts and multiple stimuli. Developmental Psychology, 24, 470-476. doi: 10.1037/0012-1649.24.4.470.
52. Meltzoff, A., & Moore, M. K. (1977). Imitation of facial and manual gestures by human neonates. Science, 198, 74-78.
53. Mervis, C. B. (1999). The Williams syndrome cognitive profile: Strengths, weaknesses, and interrelations among auditory short-term memory, language and visuo-spatial constructive cognition. In R. Fivush., W. Hirst, and E. Winigrad, (Eds.), Essays in Honor of Ulrich Neisser Mahwah, NJ: Lawrence Erlbaum.
54. Mervis, C. B., & Klein-Tasman, B. P. (2000). Williams syndrome: Cognition, personality, and adaptive behavior. Mental Retardation & Developmental Disabilities Research Reviews, 6(2), 148-158.
55. Mervis, C. B., & Robinson, B. F. (1999). Methodological issues in cross-syndrome comparisons: Matching procedures, sensitivity, and specificity. Monographs of the Society for Research in Child Development, 64, 115-131.
56. Mervis, C. B., Robinson, B. F., Bertrand, J., Morris, C. A., Klein-Tasman, B. P., & Armstrong, S. C. (2000). The Williams syndrome cognitive profile. Brain and Cognition, 44, 604-628. doi: 10.1006/brcg.2000.1232.
57. Mimura, M., Hoeft, F., Kato, M., Kobayashi, N., Sheau, K., Piggot, J., et al., (2010). A preliminary study of orbitofrontal activation and hypersociability in Williams syndrome. Journal of Neurodevelopmental Disorders, 2(2), 93-98. doi: 10.1007/s11689-009-9041-8.
58. Morris, C. A., & Mervis, C. B. (1999). Williams syndrome. In S. Goldstein., C. R. Reynolds., S. Goldstein, and C. R. Reynolds, (Eds.), Handbook of neurodevelopmental and genetic disorders in children (pp. 555-590). New York, NY US: Guilford Press.
59. Mullen, E. M. (1995). Mullen scales of early learning, AGS Edition ed.Circle Pines: American Guidance Service, Inc.: Circle Pines: American Guidance Service, Inc.
60. Mundy, P. (1995). Joint attention and social-emotional approach behavior in children with autism. Development and Psychopathology, 7(1), 63-82. doi: 10.1017/S0954579400006349.
61. Mundy, P., Sigman, M., & Kasari, C. (1990). A longitudinal study of joint attention and language development in autistic children. Journal of Autism and Developmental Disorders, 20, 115-128. doi: 10.1007/BF02206861.
62. Murray, L., & Trevarthen, C. (1986). The infant's role in mother-infant communications. Journal of Child Language, 13(1), 15-29. doi: 10.1017/S0305000900000271.
63. Nakayama, T. T., Matsuoka, R. R., Kimura, M. M., Hirota, H. H., Mikoshiba, K. K., Shimizu, Y. Y., et al., (1998). Hemizygous deletion of the HPC-1/syntaxin 1A gene (STX1A) in patients with Williams syndrome. Cytogenetics & CellGenetics, 82 (1/2), 49-51. doi: 10.1159/000015063.
64. Ninio, A., & Bruner, J. (1978). The achievement and antecedents of labeling. Journal of Child Language, 5(1), 1-15. doi: 10.1017/S0305000900001896.
65. Pezzini, G., Vicari, S., Volterra, V., Milani, L., & Ossella, M. (1999). Children with Williams syndrome: Is there a single neuropsychological profile? Developmental Neuropsychology, 15(1), 141-155. doi: 10.1080/87565649909540742.
66. Porter, M. A., Shaw, T. A., & Marsh, P. J. (2010). An unusual attraction to the eyes in Williams-Beuren syndrome: A manipulation of facial affect while measuring face scanpaths. Cognitive Neuropsychiatry, 15(6), 505-530. doi: 10.1080/13546801003644486.
67. Ratner, N., & Bruner, J. (1978). Games, social exchange and the acquisition of language. Journal of Child Language, 5(3), 391-401. doi: 10.1017/S0305000900002063.
68. Reilly, J., Klima, E. S., & Bellugi, U. (1990). Once more with feeling: Affect and language in atypical populations. Development and Psychopathology, 2(4), 367-391. doi: 10.1017/S0954579400005782.
69. Repacholi, B. M., & Gopnik, A. (1997). Early reasoning about desires: Evidence from 14- and 18-month-olds. Developmental Psychology, 33(1), 12-21. doi: 10.1037/0012-1649.33.1.12.
70. Riby, D. M., & Back, E. (2010). Can individuals with Williams syndrome interpret mental states from moving faces? Neuropsychologia, 48(7), 1914-1922. doi: 10.1016/j.neuropsychologia.2010.03.010.
71. Riby, D. M., & Hancock, P. B. (2008). Viewing it differently: Social scene perception in Williams syndrome and autism. Neuropsychologia, 46(11), 2855-28600. doi: 10.1016/j.neuropsychologia.2008.05.003.
72. Riby, D. M., & Hancock, P. B. (2009). Do faces capture the attention of individuals with Williams syndrome or autism? Evidence from tracking eye movements. Journal of Autism and Developmental Disorders, 39(3), 421-431. doi: 10.1007/s10803-008-0641-z.
73. Rogers, S. J., & Dawson, G. (2010). Early Start DenverModel for young childrenwith autism: Promoting language, learning, and engagement. New York, NY US: Guilford Press.
74. Rosner, B. A. (2002). Empathy and personal distress in young people with Williams syndrome. Dissertation Abstracts International: Section B:The Sciences and Engineering, 62 (11-B), 5390.
75. Rosner, B. A., Hodapp, R. A., Fidler, D. J., Sagun, J. N., & Dykens, E. M. (2004). Social competence in persons with Prader-Willi, Williams, and Down's syndromes. Journal of Applied Research in Intellectual Disabilities, 17, 209-217. doi: 10.1111/j.1468-3148.2004.00200.
76. Sarimski, K. (1997). Behavioural phenotypes and family stress in three mental retardation syndromes. European Child & Adolescent Psychiatry, 6(1), 26-31. doi: 10.1007/BF00573637.
77. Seibert, J. M., Hogan, A. E., & Mundy, P. C. (1982). Assessing interactional competencies: The Early Social-Communication Scales. Infant Mental Health Journal, 3(4), 244-258. doi: 10.1002/1097-0355(198224)3:4<244::AID-IMHJ2280030406>3.0.CO;2-R.
78. Sigman, M., & Ruskin, E. (1999). Continuity and change in the social competence of children with autism, Down syndrome, and developmental delays. Monographs for the Society of Research in Child Development, 64, 1-113.
79. Sparrow, S. S., Balla, D. A., & Cicchetti, D. (1998). Vineland Adaptive Behavior Scales. Circle Pines, MN: American Guidance Service.
80. Stern, D. N. (1985). The interpersonal world of the human infant. New York: Basic Books.
81. Strømme, P., Bjørnstad, P. G., & Ramstad, K. (2002). Prevalence estimation of Williams syndrome. Journal of child neurology, 17(4), 269-271. doi: 10.1177/088307380201700406.
82. Tager-Flusberg, H., Boshart, J., & Baron-Cohen, S. (1998). Reading the windows to the soul: Evidence of domain-specific sparing in Williams syndrome. Journal of Cognitive Neuroscience, 10(5), 631-639. doi: 10.1162/089892998563031.
83. Tager-Flusberg, H., & Sullivan, K. (1996). Theory of mind abilities in young children with Williams syndrome. San Francisco, CA: Cognitive Neuroscience Society.
84. Tager-Flusberg, H., & Sullivan, K. (2000). A componential view of theory of mind: Evidence from Williams syndrome. Cognition, 76(1), 59-89.
85. Tomasello, M. (1988). The role of joint attention in early language development. Language Sciences, 11, 69-88.
86. Tomasello, M. (1992). The social bases of language acquisition. Social Development, 1, 67-87. doi: 10.1111/1467-9507.ep12953134.
87. Tomasello, M., & Farrar, J. (1986). Joint attention and early language. Child Development, 57, 1454-1463. doi: 10.1111/1467-8624.ep7252234.
88. Tomc, S. A., Williamson, N. K., & Pauli, R. M. (1990). Temperament in Williams syndrome. AmericanJournal ofMedical Genetics, 36(3), 345-352.
89. Trevarthen, C. (1998). The concept and foundations of infant intersubjectivity. In S. Braten (Ed.), Intersubjective communication and emotion in early ontogeny (pp. 15-46). New York: Cambridge University Press.
90. Trevarthen, C. (2001). Infant intersubjectivity: Research, theory, and clinical applications. Journal of Child Psychology and Child Psychiatry, 42(1), 3-48.
91. Trevarthen, C. (2003). The infant's world. Journal of Child Psychology and Psychiatry, 44 (1), 154-155.
92. Udwin, O., Howlin, P., Davies, M., & Mannion, E. (1998). Community care for adults with Williams syndrome: How families cope and the availability of support networks. Journal of Intellectual Disability Research, 42(3), 238-245. doi: 10.1046/j.1365-2788.1998.00122.x.
93. Udwin, O., & Yule, W. (1991). A cognitive and behavioral phenotype in Williams syndrome. Journal of Clinical and Experimental Neuropsychology, 13(2), 232-244. doi: 10.1080/01688639108401040.
94. Uzgiris, I. C. (1989). Infants in relation: Performers, pupils, and partners. In W. Damon (Ed.), Child development today and tomorrow (pp. 288-311). San Francisco, CA: Jossey-Bass.
95. Vicari, S., Carlesimo, G., Brizzolara, D., & Pezzini, G. (1996). Short-term memory in children with Williams syndrome: A reduced contribution of lexical-semantic knowledge to word span. Neuropsychologia, 34(9), 919-925. doi: 10.1016/0028-3932(96)00007-3.
96. Wang, P. P., Doherty, S., Rourke, S. B., & Bellugi, U. (1995). Unique profile of visuoperceptual skills in a genetic syndrome. Brain and Cognition, 29(1), 54-65. doi: 10.1006/brcg.1995.1267.
97. Wang, J., Frenzel, K., Wen, D., & Falls, D. (1998). Transmembrane neuregulins interact with LIM kinase 1, a cytoplasmic protein kinase implicated in development of visuospatial cognition. The Journal of Biological Chemistry, 273(32), 20525-20534.
98. Wang, Y., Spörle, R., Paperna, T., Schughart, K., & Francke, U. (1999). Characterization and expression pattern of the frizzled gene Fzd9, the mouse homolog of FZD9 which is deleted in Williams-Beuren syndrome. Genomics, 57(2), 235-248.
99. Want, S. C., & Harris, P. L. (2002). How do children ape? Applying concepts from the study of non-human primates to the developmental study of 'imitation' in children. Developmental Science, 5(1), 1-13.
100. Wellman, H. M., Phillips, A. T., Dunphy-Lelii, S., & LaLonde, N. (2004). Infant social attention predicts preschool social cognition. Developmental Science, 7(3), 283-288. doi: 10.1111/j.1467-7687.2004.00347.x.
101. Wetherby, A. M., & Pruting, C. A. (1984). Profiles of communicative and cognitivesocial abilities in autistic children. Journal of Speech and Hearing Research, 27(3), 364-377.
102. Whalen, C., & Schreibman, L. (2003). Joint attention training for children with autism using behavior modification procedures. Journal of Child Psychology and Psychiatry, 44 (3), 456-468.
103. Williams, J. C. P., Barrat-Boyers, B. G., & Lowe, J. B. (1961). Supravalvular aortic stenosis. Circulation, 24, 1311-1318.