Neuropsychological profile of Italians with Williams syndrome: An example of a dissociation between language and cognition?

Journal of the International Neuropsychological Society

Volumn 10, Issue 6, 2004, Pages 862-876

  Vicari, Stefano ^a,b,c   Bates, Elisabeth ^d   Caselli, Maria Cristina ^b,c   Pasqualetti, Patrizio ^e   Gagliardi, Chiara ^f   Tonucci, Francesca ^a   Volterra, Virginia ^b  

^a BAMBINO GESÙ CHILDREN S HOSPITAL   (Italy)

^b CNR   (Italy)

^c LUMSA UNIVERSITY   (Italy)

^d UNIVERSITY OF CALIFORNIA   (United States)

^e IRCCS ISTITUTO CENTRO SAN GIOVANNI DI DIO FATEBENEFRATELLI   (Italy)

^f SCIENTIFIC INSTITUTE   (Italy)

Author keywords

Cognitive impairment; Developmental trajectories; Genetic syndromes

Indexed keywords

ADOLESCENT; AGE; ANALYSIS OF VARIANCE; ARTICLE; CHILD; COGNITION; CONTROLLED STUDY; DEPTH PERCEPTION; DISSOCIATION; DOWN SYNDROME; HUMAN; INTELLIGENCE QUOTIENT; ITALY; LANGUAGE; LONGITUDINAL STUDY; MAJOR CLINICAL STUDY; NEUROPSYCHOLOGY; NONVERBAL COMMUNICATION; PHONETICS; PRIORITY JOURNAL; SEMANTICS; TASK PERFORMANCE; WILLIAMS BEUREN SYNDROME;

ADOLESCENT; ADULT; CHILD; CHILD, PRESCHOOL; COGNITION; FEMALE; HUMANS; INTELLIGENCE TESTS; ITALY; LANGUAGE; MALE; MEMORY; MEMORY, SHORT-TERM; NEUROPSYCHOLOGICAL TESTS; PHENOTYPE; PSYCHOLINGUISTICS; SPACE PERCEPTION; VERBAL BEHAVIOR; WECHSLER SCALES; WILLIAMS SYNDROME;

EID: 8344287489     PISSN: 13556177     EISSN: None     Source Type: Journal    
DOI: 10.1017/S1355617704106073     Document Type: Article

References (68)

1. Arnold, R., Yule, W., & Martin, N. (1985). The psychological characteristics of infantile hypercalcaemia: A preliminary investigation. Developmental Medicine and Child Neurology, 27, 49-59.
2. Atkinson, J., Braddick, O., Anker, S., Curran, W., Andrew, R., Wattam-Bell, J., & Braddick, F. (2003). Neurobiological models of visuospatial cognition in children with Williams syndrome: Measures of dorsal-stream and frontal function. Developmental Neuropsychology, 23 139-172.
3. Atkinson, J., King, J., Braddick, O., Nokes, L., Anker, S., & Braddick, F. (1997). A specific deficit of dorsal stream function in Williams Syndrome. Neuroreport, 8, 1919-1922.
4. Atkinson, J., Shirley, L., Braddick, O., Mason, A., & Braddick, F. (2001). Visual and visuospatial development in young children with Williams syndrome. Developmental Medicine and Child Neurology, 43, 330-337.
5. Baddeley, A D. (1986). Working memory. Oxford, UK: Oxford University Press.
6. Baddeley, A.D. & Hitch, G. (1974). Working memory. In G.H. Bower (Ed.), The psychology of learning and motivation (pp. 47-90 . New York: Academic Press.
7. Beery, K. (1997). Developmental tests of visual-motor integration. Parsippany, NJ: Modern Curriculum Press.
8. Bellugi, U., Bihrle, A., Jernigan, T., Trauner, D., & Doherty S. (1990). Neuropsychological, neurological and neuroanatomical profile of Williams Syndrome. American Journal of Medical Genetics (Suppl. 6), 115-125.
9. Bellugi, U., Bihrle, A., Neville, H., Jernigan, T., & Doherty, S. (1996). Language, cognition and brain organization in a neurodevelopmental disorder. In M. Gunnar & C. Nelson (Eds.), Developmental behavioral neuroscience. Hillsdale, NJ: Erlbaum.
10. Bellugi, U., Koremberg, J.R., & Klima, E.S. (2001). Williams syndrome: An exploration of neurocognitive and genetic features. Clinical Neuroscience Research, 1, 217-229.
11. Bellugi, U., Lichtenberger, L., Mills, D., Galaburda, A., & Koremberg, J.R. (1999a). Bridging cognition, the brain and molecular genetics: Evidence from Williams syndrome. Trends in Neuroscience, 22 197-207.
12. Bellugi, U., Mills, D., Jernigan, T., Hickok, G., & Galaburda, A. (1999b). Linking cognition, brain structure, and brain function in Williams syndrome. In H. Tager-Flusberg (Ed.), Neurodevelopmental disorders (pp. 111-136). Cambridge, MA: MIT Press.
13. Bellugi, U. & St. George, M. (Eds). (2001). Journey from cognition to brain to gene. Perspectives from Williams syndrome. Cambridge MA: MIT Press.
14. Botta, A., Sangiuolo, F., Calza, L., Giardino, L., Potenza, S., Novelli, G., & Dallapiccola, B. (1999). Expression analysis and protein localization of the human HPC-1/syntaxin 1A, a gene deleted in Williams syndrome. Genomics, 15, 525-528.
15. Bozzo, M.T. & Mansueto Zecca, G. (1993). Adattamento italiano della Scala d'Intelligenza Stanford-Binet Forma L-M nella revisione Terman-Merill [Italian adaptation of the Stanford-Binet Intelligence Scale, Form L-M in the Terman-Merill revision]. Firenze, Italy: Organizzazioni Speciali.
16. Chapman, R.S. (1995). Language development in children and adolescents with Down syndrome. In P. Fletcher & B. MacWhinney (Eds. , Handbook of child language (pp. 641-663). Oxford, UK: Blackwell.
17. Courtney, S.M., Ungerleider, L.G., Keil, K., & Haxby, J.V. (1996). Object and visual working memory activate separate neural systems in human cortex. Cerebral Cortex, 6, 39-49.
18. Devescovi, A. & Caselli, M.C. (2001). Una prova di ripetizione di frasi per la valutazione del primo sviluppo grammaticale [A test of phrase repetition to evaluate the early development of grammar]. Psicologia Clinica dello Sviluppo, 3, 341-364.
19. Devescovi, A., Caselli, M.C., Ossella, T., & Alviggi, F.G. (1992). Strumenti di indagine sulle prime fasi dello sviluppo linguistico: Risultati di una prova di ripetizione di frasi con bambini fra i due e i tre anni e mezzo [Methods of enquiry into the early phases in linguistic development: Results of a sentence repetition test in 2-3.5-year-old children]. Rassegna di Psicologia, 2, 29-54.
20. Dunn, Lloyd M. & Dunn, Leota M. (1981). Peabody Picture Vocabulary Test-Revised. Circle Pines, MN: American Guidance Service.
21. Ewart, A.K., Morris, C.A., Atkinson, D., Jin, W., Sternes, K., Spallone, P., Stock, A.D., Leppert, M., & Keating, M.T. (1993). Hemizygosity at the elastin locus in a developmental disorder, Williams syndrome. Nature Genetics, 5, 11-16.
22. Fabbretti, D., Pizzuto, E., Vicari, S., & Volterra, V. (1997). A story description task in children with Down syndrome: Lexical and morphosyntactic abilities. Journal of Intellectual Disabilities Research, 41, 165-179.
23. Fabbro, F., Alberti, A., Gagliardi, C., & Borgatti, R. (2002). Differences in native and foreign language repetition task between subjects with Williams and Down syndromes. Journal of Neurolinguistics, 15, 1-10.
24. Frangiskakis, J.M., Ewart, A.K., Morris, C.A., Mervis, C.B., Bertrand, J., Robinson, B.F., Klein, B.P., Ensing, G.J., Everett, L.A., & Green, E.D. (1996). LIM-Kinase-1 hemizygosity implicated in impaired visuospatial constructive cognition. Cell, 86, 59-69.
25. Gosh, A., Stading, G., & Pankau, R. (1994). Linguistic abilities in children with Williams-Beuren syndrome. American Journal of Medical Genetics, 52, 291-296.
26. Grant, J., Karmiloff-Smith, A., Gathercole, S.E., Paterson, S., Howlin, P., Davies, M., & Udwin, O. (1997). Phonological short-term memory and its relationship to language in Williams Syndrome. Journal of Cognitive Neuropsychiatry, 2, 81-99.
27. Jarrold, C., Baddeley, A.D., & Hewes, A.K.(1999) Genetically dissociated components of working memory: Evidence from Down and Williams syndrome. Neuropsychologia, 37, 637-651.
28. Jernigan, T.L., Bellugi, U., Sowell, E., Doherty, S., & Hesselink, J.R. (1993). Cerebral morphologic distinction between Williams and Down syndromes. Archives of Neurology, 50, 186-191.
29. Karmiloff-Smith, A. (1998). Development itself is the key to understanding developmental disorders. Trends in Cognitive Sciences, 2 289-298.
30. Karmiloff-Smith, A., Brown, J.H., Grice, S., & Paterson, S. (2003). Dethroning the myth: Cognitive dissociations and innate modularity in Williams syndrome. Developmental Neuropsychology, 23, 227-242.
31. Karmiloff-Smith, A., Grant, J., Berthoud, I., Davis, M., Howlin, P., & Udwin, O. (1997). Language and Williams syndrome: How intact is "intact"? Child Development, 68, 246-262.
32. Karmiloff-Smith, A., Tyler, L.K., Voice, K., Sims, K., Udwin, O., Howlin, P., & Davis, M. (1998). Linguistic dissociations in Williams syndrome: Evaluating receptive syntax in on-line and off-line tasks. Neuropsychologia, 36, 343-351.
33. Klein, B.P. & Mervis, C.B. (1999). Cognitive strength and weaknesses of 9- and 10-year-olds with Williams syndrome or Down syndrome. Developmental Neuropsychology, 16, 177-196.
34. Majerus, S., Barisnikov, K., Vuillemin, I., Poncelet, M., & Van der Linden, M. (in press). An investigation of verbal short-term memory and phonological processing in four children with Williams syndrome. Neurocase.
35. Mervis, C.B., Morris, C.A., Bertrand, J., & Robinson, B.F. (1999). Williams syndrome: Findings from an integrated program of research. In H. Tager-Flusberg (Ed.), Neurodevelopmental disorders (pp. 65-110). Cambridge, MA: MIT Press.
36. Mervis, C.B. & Robinson, B.R. (2000). Cognitive strength and weaknesses of 9- and 10-year-olds with Williams syndrome or Down syndrome. Developmental Neuropsychology, 16, 177-196.
37. Miller, J.F. (1988). The developmental asynchrony of language development in children with Down syndrome. In L. Nadel (Ed.), Psychobiology of Down syndrome (pp. 167-198). Cambridge, MA: MIT Press.
38. Miller, J.F. ( 1992). Development of speech and language in children with Down syndrome. In I.T. Lott & E.E. McLoy (Eds.), Down syndrome (pp. 39-50). New York: Wiley-Liss.
39. Nelson, C.A., Monk, C.S., Lin, J., Carver, L.J., Thomas, K.M., & Truwit, C.L. (2000). Functional neuroanatomy of spatial working memory in children. Developmental Psychology, 36, 109-116.
40. Nicholas, L., Brookshire, R., Mc Lennan, D., Schumacher, J., & Porrazzo, S. (1989). Revised administration and scoring procedures for the Boston Naming Test and norms for non-brain-damaged adults. Aphasiology, 3, 569-580.
41. Orsini, A., Grossi, D., Capitani, E., Laiacona, M., Papagno, C., & Vallar, G. (1987). Verbal and spatial immediate memory span: Normative data from 1355 adults and 1112 children. Italian Journal of Neurological Sciences, 6, 539-548.
42. Papagno, C. & Vallar, G. (2001). Understanding metaphors and idioms: A single-case neuropsychological study in a person with Down syndrome. Journal of the International Neuropsychological Society, 7 516-528.
43. Paterson, S., Brown, J.H., Gsodl, M.K., Johnson, M.C., & Karmiloff-Smith, A. (1999, December). Cognitive modularity and genetic disorders. Science, 286, 2355-2358.
44. Pezzini, G., Vicari S., Volterra V., Milani L., & Ossella M.T. (1999). Children with Williams syndrome: Is there a unique neuropsychological profile? Developmental Neuropsychology, 15, 141-155.
45. Piattelli-Palmarini, M. (2001). Speaking of learning. Nature, 411, 887-888.
46. Rae, C., Karmiloff-Smith, A., Lee, M.A., Dixon, R.M, Grant, J., Blamire, A.M., Thompson, C.H., Styles, P., & Radda, G.K. (1998). Brain biochemistry in Williams syndrome: Evidence for a role of the cerebellum in cognition. Neurology, 51, 33-40.
47. Reilly, J., Klima, E.S., & Bellugi, U. (1990). Once more with feeling: Affect and language in atypical populations. Development and Psychopathology, 2, 367-391.
48. Reiss, A.L., Eliez, S., Schmitt, J.E., & Straus, E. (2000). Neuroanatomy of Williams syndrome: A high-resolution MRI study. Journal of Cognitive Neuroscience, 12, 65-73.
49. Riva, D., Nichelli, F., & Devoti, M. (2000). Developmental aspects of verbal fluency and confrontation naming in children. Brain and Language, 71, 267-284.
50. Rondal, J.A. (1995). Exceptional language development in Down syndrome. Cambridge, UK: Cambridge University Press.
51. Rondal, J.A., Ghiotto, M., Brédart, S., & Bachelet, J.E (1988). Mean length of utterance of children with Down syndrome. American Journal on Mental Retardation, 93, 64-66.
52. Rubba, J. & Klima, E.S. (1991). Preposition use in a speaker with Williams Syndrome: Some cognitive grammar proposals. Center for Research in Language Newsletter, 3, 3-12.
53. Rustioni, D. (1994). Sviluppo della comprensione linguistica nei bambini italiani. Presentazione di una scala evolutiva [Development of grammar comprehension in Italian children. Presentation of an evolutionary scale]. In C. Cornoldi & R. Vianello (Eds.), Handicap e apprendimento (pp. 35-53). Bergamo, Italy: Junior Ed.
54. Singer-Harris, N.G., Bellugi, U., Bates, E., Jones, W., & Rossen, M. (1997). Contrasting profiles of language in children with Williams and Down syndromes. Developmental Neuropsychology, 13, 345-370.
55. Tomaiuolo, F., Di Paola, M., Caravale, B., Vicari, S., Petrides, M., & Caltagirone C. (2002). Morphology and morphometry of the corpus callosum in Williams syndrome: A magnetic resonance imaging analysis. Neuroreport, 13, 1-5.
56. Udwin, O. & Yule, W. (1990). Expressive language of children with Williams syndrome. American Journal of Medical Genetics (Suppl. 6), 108-114.
57. Vallar, G. & Papagno, C. (1993). Preserved vocabulary acquisition in Down syndrome children: The role of phonological short-term memory. Cortex, 29, 467-483.
58. Vender, C., Borgia, R., Bruno, S., Freo, P., & Zardini, G. (1981). Un test di ripetizione di frasi. Analisi delle performances in bambini normali [A test of sentence repetition. Analysis of performance in normal children]. Giornale di Neuropsichiatria Infantile, 243-244, 819-831.
59. Vicari, S., Bellucci, S., & Carlesimo, G.A. (2001). Procedural learning deficit in children with Williams syndrome. Neuropsychologia, 39, 665-677.
60. Vicari, S., Bellucci, S., & Carlesimo, G.A. (2003). Visual and spatial working memory dissociation: Evidence from a genetic syndrome. Developmental Medicine and Child Neurology, 45, 269-273.
61. Vicari, S., Brizzolara, D., Carlesimo, A., Pezzini, G., & Volterra, V. (1996a). Memory abilities in children with Williams syndrome. Cortex, 32, 503-514.
62. Vicari, S., Carlesimo, A., Brizzolara, D., & Pezzini, G. (1996b). Short-term memory in children with Williams syndrome: A reduced contribution of lexical-semantic knowledge to word span. Neuropsychologia, 34, 919-925.
63. Vicari, S., Caselli, M.C., Gagliardi, C., Tonucci, F., & Volterra, V. (2002). Language acquisition in special populations: A comparison between Down and Williams syndromes. Neuropsychologia, 40 2461-2470.
64. Vicari, S., Caselli, M.C., & Tonucci, F. (2000). Asynchrony of lexical and morphosyntactic development in children with Down syndrome. Neuropsychologia, 38, 634-644.
65. Volterra, V., Capirci, O., Pezzini, G., Sabbadini, L., & Vicari, S. (1996). Linguistic abilities in Italian children with Williams syndrome. Cortex, 32, 663-677.
66. Volterra, V., Longobardi, E., Pezzini, G., Vicari, S., & Antenore, C. (1999). Visuo-spatial and linguistic abilities in a twin with Williams syndrome. Journal of Intellectual Disability Research, 43, 294-305.
67. Wang, P.P. & Bellugi, U. (1994). Evidence from two genetic syndromes for dissociation between verbal and visual-spatial short-term memory. Journal of Clinical Experimental Neuropsychology, 16, 317-322.
68. Wechsler, D. (1986). WISC-R-Scala di intelligenza Wechsler per bambini, riveduta [WISC-R-Wechsler Intelligence Scale for Children, revised]. Florence, Italy: Organizzazioni Speciali.