Williams syndrome: Cognition, personality, and adaptive behavior

Mental Retardation and Developmental Disabilities Research Reviews

Volumn 6, Issue 2, 2000, Pages 148-158

  Mervis, Carolyn B ^a   Klein Tasman, Bonita P ^b  

^a UNIVERSITY OF LOUISVILLE   (United States)

^b EMORY UNIVERSITY   (United States)

Author keywords

Adaptive behavior; Cognition; Genetic syndrome; Genotype phenotype correlations; Language; Memory; Personality; Visuo spatial construction; Williams syndrome

Indexed keywords

ADAPTIVE BEHAVIOR; COGNITIVE DEVELOPMENT; DEPTH PERCEPTION; HUMAN; INTELLIGENCE QUOTIENT; LANGUAGE DEVELOPMENT; MEMORY; PERSONALITY; PRIORITY JOURNAL; REVIEW; TEMPERAMENT; WILLIAMS BEUREN SYNDROME;

ADAPTATION, PSYCHOLOGICAL; BEHAVIOR; COGNITION; HUMANS; INTELLIGENCE; PERSONALITY; WILLIAMS SYNDROME;

EID: 0034043239     PISSN: 10804013     EISSN: None     Source Type: Journal    
DOI: 10.1002/1098-2779(2000)6:2<148::AID-MRDD10>3.0.CO;2-T     Document Type: Review

References (78)

1. Achenbach TM. 1991. Child behavior checklist for ages 4-18. Burlington, VT: University of Vermont Press.
2. Ashkoomoff NA, Stiles J. 1996. The influence of pattern type on children's block design performance. J Int Neuropsychol Soc 2:392-402.
3. Atkinson J, Braddick O, Anker S, et al. 2000. Neurobiological models of visuospatial cognition in young children with Williams syndrome: Measures of dorsal stream and frontal function. Dev Neuropsychol. In press.
4. Bayley N. 1969. Bayley scales of infant development. New York: Psychological Corporation.
5. Bayley N. 1993. Bayley scales of infant development. 2nd edition. San Antonio, TX: Psychological Corporation.
6. Beery KE. 1989. Developmental test of visual-motor integration. 3rd revision. Cleveland: Modern Curriculum Press.
7. Bellugi U, Marks S, Bihrle A, et al. 1988. Dissociation between language and cognitive functions in Williams syndrome. In Bishop D and Mogford K, editors. Language development in exceptional circumstances. Edinburgh: Churchill Livingstone, p 177-189.
8. Bellugi U, Mills D, Jernigan T, et al. 1999. Linking cognition, brain structure, and brain function in Williams syndrome. In Tager-Flusberg H, editor. Neurodevelopmental disorders. Cambridge, MA: The MIT Press, p 111-136.
9. Bellugi U, Wang PP, Jernigan TL. 1994. Williams syndrome: An unusual neuropsychological profile. In Broman SH, Grafman J, editors. Atypical cognitive deficits in developmental disorders: Implications for brain function. Hillsdale, NJ: Erlbaum, p 23-56.
10. Bertrand J, Mervis CB. 1996. Longitudinal analysis of drawings by children with Williams syndrome: Preliminary results. Visual Arts Res 22:19-34.
11. Bertrand J, Mervis CB, Eisenberg JD. 1997. Drawing by children with Williams syndrome: A developmental perspective. Dev Neuropsychol, 13:41-67.
12. Bregman JD. 1996. Social, affective, and behavioral impairments in Williams syndrome. Paper presented at the National Professional Meeting of the Williams Syndrome Association. Valley Forge, PA.
13. Chapman RS. 1997. Language development in children and adolescents with Down syndrome. Ment Retard Develop Disabil Res Rev 3:307-312.
14. Davies M, Udwin O, Howlin P. 1998. Adults with Williams syndrome: Preliminary study of social, emotional, and behavioural difficulties. Br J Psychiatry 172:273-276.
15. Derryberry D, Rothbart MK. 1997. Reactive and effortful processes in the organization of temperament. Dev Psychopath 9:633-652.
16. Dilts CV, Morris CA, Leonard CO. 1990. Hypothesis for development of a behavioral phenotype in Williams syndrome. Am J Med Genet Suppl 6:126-131.
17. Dunn LE, Dunn LE. 1981. Peabody Picture Vocabulary Test-Revised. Circle Pines, MN: American Guidance Services.
18. Dykens EM, Rosner BA. 1999. Refining behavioral phenotypes: Personality-motivation in Williams and Prader-Willi syndromes. Am J Ment Retard 104: 158-169.
19. Einfeld SL, Tonge BJ, Florio T. 1997. Behavioral and emotional disturbance in individuals with Williams syndrome. Am J Ment Retard 102: 45-53.
20. Elliott CD. 1990. Differential ability scales. San Diego, CA: Harcourt Brace Javanovich.
21. Ewart AK, Morris CA, Atkinson D, et al. 1993. Hemizygosity at the elastin locus in a developmental disorder, Williams syndrome. Nat Genet 5:11-16.
22. Fenson L, Dale PS, Reznick JS, et al. 1993. MacArthur Communicative Development Inventories: User's guide and technical manual. San Diego, CA: Singular.
23. Frangiskakis JM, Ewart AK, Morris CA, et al. 1996. LIM-Kinase1 hemizygosity implicated in impaired visuospatial constructive cognition. Cell 86:59-69.
24. Golinkoff RM, Mervis CB, Hirsh-Pasek K. 1994. Early object labels: The case for a developmental lexical principles framework. J Child Lang 21:125-155.
25. Gosch A, and Pankau R. 1994. Social-emotional and behavioral adjustment in children with Williams-Beuren syndrome. Am J Med Genet, 53:335-339.
26. Gosch A, Pankau R. 1997. Personality characteristics and behaviour problems in individuals of different ages with Williams syndrome. Dev Med Child Neurol 39:327-533.
27. Gosch A, Stading G, Pankau R. 1994. Linguistic abilities in children with Williams-Beuren syndrome. Am J Med Genet 52:291-296.
28. Greer MK, Brown FR, Pai GS, et al. 1997. Cognitive, adaptive, and behavioral characteristics of Williams syndrome. Am J Med Genet 74: 521-525.
29. Jarrold C, Baddeley AD, Hewes AK. 1998. Verbal and nonverbal abilities in the Williams syndrome phenotype: evidence for diverging developmental trajectories. J Child Psychol Psychiatry 39:511-523.
30. Jarrold C, Baddeley AD, Hewes AK. 1999. Genetically dissociated components of working memory: evidence from Down's and Williams syndrome. Neuropsychologia 37:637-651.
31. Karmiloff-Smith A, Grant J, Berthoud I, et al. 1997. Language and Williams syndrome: How intact is "intact"? Child Dev 68:246-262.
32. Karmiloff-Smith A, Klima E, Bellugi U, et al. 1995. Is there a social module? Language, face processing and theory of mind in individuals with Williams syndrome. J Cogn Neurosci 7:196-208.
33. Kaufman AS, Kaufman NL. 1990. Kaufman Brief Intelligence Test. Circle Pines, MN: American Guidance Service.
34. Klein BP, Mervis CB. 1999. Contrasting patterns of cognitive abilities of 9-and 10-year-olds with Williams syndrome or Down syndrome. Dev Neuropsychol 16:177-196.
35. Klein-Tasman BP, Mervis CB. in press. Distinctive personality profile of children with Williams syndrome. Dev Neuropsychol.
36. Landau B, Zukowski A. 2000. Objects, motions, and paths: Spatial language of children with Williams syndrome. Dev Neuropsychol. In press.
37. Leonard LB. 1998. Children with specific language impairment. Cambridge: The MIT Press.
38. Leonard LB. 1998. Children with Specific Language Impairment. Cambridge, MA: MIT Press.
39. Levine G, Parkinson S. 1994. Detection, discrimination, and the theory of signal detection. In: Levine G, Parkinson S, editors. Experimental Methods in Psychology. Hillsdale, NJ: Erlbaum, 204-233.
40. McCarthy D. 1972. McCarthy Scales of Children's Abilities. New York: Psychological Corporation.
41. Mervis CB. 1999. The Williams syndrome cognitive profile: Strengths, weaknesses, and interrelations among auditory short term memory, language, and visuospatial constructive cognition. In Winograd E. Fivush R, Hirst W, editors. Ecological approaches to cognition: Essays in honor of Ulric Neisser. Mahwah, NJ: Erlbaum, p 193-227.
42. Mervis CB, Bertrand J. 1997. Developmental relations between cognition and language: Evidence from Williams syndrome. In Adamson LB, Romski MA, editors. Communication and language acquisition: Discoveries from atypical development. Baltimore: Paul H, Brookes, p 75-106.
43. Mervis CB, Klein BP, and Rothstein M. 1999a. Adaptive behavior of 4-through 8-year-old children with Williams syndrome. Manuscript under review.
44. Mervis CB, Morris CA, Bertrand J, et al. 1999b. Williams syndrome: Findings from an integrated program of research. In Tager-Flusberg H, editor. Neurodevelopmental disorders Cambridge, MA: The MIT Press, p 65-110.
45. Mervis CB, Morris CA, Klein-Tasman BP, et al. 2000. Attentional characteristics of infants and toddlers with Williams syndrome when interacting with other individuals. Dev Neuropsychol. In press.
46. Mervis CB, Robinson BF, 2000. Early expressive-vocabularies of children with Williams syndrome or Down syndrome: A comparison. Dev Neuropsychol. In press.
47. Mervis CB, Robinson BF, Bertrand J, et al. 1998. The Williams syndrome cognitive profile. Manuscript under review.
48. Mervis CB, Robinson BF, Pani JR. 1999. Visuospatial construction. Am J Hum Genet 65: 1222-1229.
49. Morris CA, Mervis CB. 1999. In Goldstein S, Reynolds CR, editors. Handbook of neurodevelopmental and genetic disorders in children. New York: Guilford, p 555-590.
50. Morris CA, Mervis CB. 2000. Williams syndrome and related disorders. Ann Rev Genomics Hum Genet 1. In press.
51. Mullen EM. 1995. Mullen Scales of Early Learning. Circle Pines, MN: American Guidance Service.
52. Pagon RA, Bennett FC, LaVeck, B. et al. 1987. Williams syndrome: Features in late childhood and adolescence. Pediatrics 80:85-91.
53. Pani JR, Mervis CB, Robinson BF. 1999. Global spatial organization by individuals with Williams syndrome. Psychological Science 10:453-458.
54. Paterson SJ, Brown JH, Gsodl MK. et al. 1999. Cognitive modularity and genetic disorders. Science 286:2355-2358.
55. Rice ML, Mervis CB, Klein BP, et al. 1999. Morphological abilities of children with specific language impairment or Williams syndrome. Paper presented at the Boston University Conference on Language Development, Boston, MA.
56. Rice ML, Wexler K. 1996. A phenotype of specific language impairment: Extended optional infinitives. In Rice ML, editor. Toward a genetics of language. Mahwah, NJ: Erlbaum, p 215-237.
57. Rothbart MK, Ahadi SA. 1994. Temperament and the development of personality. J Abnorm Psychol 103:55-66.
58. Rothbart MK, Ahadi SA, Hershey KL. 1994. Temperament and social behavior in childhood. Merrill-Palmer Quarterly 40:21-39.
59. Sarimski K. 1997. Behavioural phenotypes and family stress in three mental retardation syndromes. Eur J Child Adolesc Psychiatry 6:26-31.
60. Sparrow SS, Balla DA, Cicchetti DV. 1984. Vineland Adaptive Behavior Scales-Interview Edition. Circle Pines, MN: American Guidance Service.
61. Sullivan K, Tager-Flusberg H. 1999. Second-order belief attribution in Williams syndrome: Intact or impaired? Am J Ment Retard 104: 523-532.
62. Sullivan K, Tager-Flusberg H. 2000. Can adolescents with Williams syndrome tell the difference between lies and jokes? Dev Neuropsychol. In press.
63. Sullivan K, Winner E, Hopfield N. 1995. How children tell a lie from a joke: The role of second-order mental attributions. Br J Dev Psychol 13:191-204.
64. Sullivan K, Zaitchik D, Tager-Flusberg H. 1994. Preschoolers can attribute second-order beliefs. Dev Psychol 30:395-402.
65. Swets JA. 1988. Measuring the accuracy of diagnostic systems. Science 240:1285-1293.
66. Tager-Flusberg H, Boshart J, Baron-Cohen S. 1998. Reading the windows to the soul: Evidence of domain-specific sparing in Williams syndrome. J Cogn Neurosci 10:631-639.
67. Tager-Flusberg H, Sullivan K. 1999. Are children with Williams syndrome spared in theory of mind? Paper presented at the biennial meeting of the Society for Research in Child Development, Albuquerque, NM.
68. Tager-Flusberg H, Sullivan K. 2000. A componential view of theory of mind: Evidence from Williams syndrome. Cognition. In press.
69. Tellegen A. 1985. Structures of mood and personality and their relevance to assessing anxiety, with an emphasis on self-report. In Tuma AH, Maser JD, editors. Anxiety and the anxiety disorders Hillsdale, NJ: Erlbaum, p 681-716.
70. Thomas SA, Chess S. 1977. Temperament and development. New York: Bruner/Mazel.
71. Tome SA, Williamson NK, Pauli RM. 1990. Temperament in Williams syndrome. Am J Med Genet 36:345-352.
72. Udwin, O, Yule W. 1990. Expressive language of children with Williams syndrome. Am J Med Genet Suppl 6:108-114.
73. Udwin O, Yule W. 1991. A cognitive and behavioral phenotype in Williams syndrome. J Clin Exp Neuropsychol 13:232-244.
74. Volterra V, Capirci O, Pezzini G, et al. 1996. Linguistic abilities m Italian children with Williams syndrome. Cortex 32:663-677.
75. Von Arnim G, Engel P. 1964. Mental retardation related to hypercalcaemia. Dev Med Child Neurol 6:366-377.
76. Wang PP, Bellugi U. 1994. Evidence from two genetic syndromes for a dissociation between verbal and visual-spatial short-term memory. J Clin Exp Neuropsychol 16:317-322.
77. Wang PP, Doherty S, Rourke SB, et al. 1995. Unique profile of visuo-perceptual skills in a genetic syndrome. Brain Cogn 29:54-65.
78. Wilson B, Cockburn J, Baddeley A. 1985. Rivermead Behavioural Memory Test. Reading, England: Thames Valley Test.