Executive functions in individuals with Williams syndrome

Journal of Intellectual Disability Research

Volumn 54, Issue 5, 2010, Pages 418-432

  Menghini, D ^a,b   Addona, F ^a   Costanzo, F ^a   Vicari, S ^a  

^a BAMBINO GESÙ CHILDREN S HOSPITAL   (Italy)

^b EUROPEAN UNIVERSITY OF ROME   (Italy)

Author keywords

Behavioural phenotypes; Genetic syndrome; Intellectual disability; Neuropsychological profile

Indexed keywords

ADOLESCENT; ATTENTION; CHILD; COGNITION DISORDERS; EXECUTIVE FUNCTION; FEMALE; HEARING; HUMAN; INHIBITION (PSYCHOLOGY); LEARNING; MALE; NEUROPSYCHOLOGICAL TEST; ORIENTATION; PATTERN RECOGNITION; PERCEPTION; PROBLEM SOLVING; PSYCHOLOGY; PSYCHOMETRY; PSYCHOMOTOR PERFORMANCE; REACTION TIME; REFERENCE VALUE; SHORT TERM MEMORY; STATISTICS AND NUMERICAL DATA; STROOP TEST; WILLIAMS SYNDROME;

ADOLESCENT; ASSOCIATION LEARNING; ATTENTION; AUDITORY PERCEPTION; CHILD; COGNITION DISORDERS; EXECUTIVE FUNCTION; FEMALE; HUMANS; INHIBITION (PSYCHOLOGY); MALE; MEMORY, SHORT-TERM; NEUROPSYCHOLOGICAL TESTS; ORIENTATION; PATTERN RECOGNITION, VISUAL; PROBLEM SOLVING; PSYCHOMETRICS; PSYCHOMOTOR PERFORMANCE; REACTION TIME; REFERENCE VALUES; REVERSAL LEARNING; SET (PSYCHOLOGY); SIZE PERCEPTION; STROOP TEST; VERBAL LEARNING; WILLIAMS SYNDROME;

EID: 77953352174     PISSN: 09642633     EISSN: 13652788     Source Type: Journal    
DOI: 10.1111/j.1365-2788.2010.01287.x     Document Type: Article

References (78)

1. Altemeier L E, Abbott R D, Berninger V W. Executive functions for reading and writing in typical literacy development and dyslexia. Journal of Clinical and Experimental Neuropsychology 2008, 30:588-606.
2. Anderson V, Levin H S, Jacobs R. Executive functions after frontal lobe injury: a developmental perspective. Principles of Frontal Lobe Function 2002, 504-27. Stuss D T, Knight R T, In, eds, pp., Oxford University Press, London
3. Arnold R, Yule W, Martin N. The psychological characteristics of infantile hypercalcaemia: a preliminary investigation. Developmental Medicine and Child Neurology 1985, 27:49-59.
4. Atkinson J. The Developing Visual Brain 2000, Oxford University Press, Oxford
5. Atkinson J, King J, Braddick O, Nokes L, Anker S, Braddick F. A specific deficit of dorsal stream function in William's syndrome. Neuroreport: An International Journal for the Rapid Communication of Research in Neuroscience 1997, 8:1919-22.
6. Atkinson J, Braddick O, Anker S, Curran W, Andrew R, Wattam-Bell J. Neurobiological models of visuospatial cognition in children with Williams syndrome: measures of dorsal-stream and frontal function. Developmental Neuropsychology 2003, 23:139-72.
7. Baddeley A D, Hitch G J. Working memory. Recent Advances in Learning and Motivation 1974, 47-90. Bower G A, In, ed., pp., Academic Press, New York, NY
8. Baddeley A D, Logie R H. Working memory: the multiple-component model. Models of Working Memory: Mechanisms of Active Maintenance and Executive Control 1999, 28-61. Miyake A, Shah P. In, eds, pp., Cambridge University Press, Cambridge
9. Barkley R A, Edwards G, Laneri M, Fletcher K. Executive functioning, temporal discounting, and sense of time in adolescents with attention deficit hyperactivity disorder (ADHD) and oppositional defiant disorder (ODD). Journal of Abnormal Child Psychology 2001, 29:541-56.
10. Bellugi U, Bihrle A, Jernigan T, Trauner D, Doherty S. Neuropsychological, neurological, and neuroanatomical profile of Williams syndrome. American Journal of Medical Genetics 1990, 6:115-25.
11. Bellugi U, Lichtenberger L, Jones W, Lai Z. The neurocognitive profile of Williams syndrome: a complex pattern of strengths and weaknesses. Journal of Cognitive Neuroscience 2000, 12:7-29.
12. Bellugi U, Lichtenberger L, Jones W, Lai Z, George MSt. The neurocognitive profile of Williams syndrome: a complex pattern of strengths and weaknesses. Journey from Cognition to Brain to Gene: Perspectives from Williams Syndrome 2001, 1-41. Bellugi U, George MSt, In, eds, pp., MIT Press, Cambridge, MA
13. Biancardi A, Stoppa E. Il Test delle Campanelle modificato: una proposta per lo studio dell'attenzione in età evolutiva. Psichiatria dell'Infanzia e dell'Adolescenza 1997, 64:73-84.
14. Bisiacchi P, Cendron M, Gugliotta M, Tressoldi P E, Vio C. BVN 5-11 Batteria Di Valutazione Neuropsicologica Per L'età Evolutiva 2005, Erickson, Trento
15. Brown J H, Johnson M H, Paterson S J, Gilmore R, Longhi E, Karmiloff-Smith A. Spatial representation and attention in toddlers with Williams syndrome and Down syndrome. Neuropsychologia 2003, 41:1037-46.
16. Carrasco X, Castillo S, Aravena T, Rothhammer P, Aboitiz F. Williams syndrome: pediatric, neurologic, and cognitive development. Pediatric Neurology 2005, 32:166-72.
17. Corbett B A, Constantine L J, Hendren R, Rocke D, Ozonoff S. Examining executive functioning in children with autism spectrum disorder, attention deficit hyperactivity disorder and typical development. Psychiatry Research 2009, 166:210-22.
18. Cornish K, Scerif G, Karmiloff-Smith A. Tracing syndrome-specific trajectories of attention across the lifespan. Cortex 2007, 43:672-85.
19. Cornoldi C, Vecchi T. Visuo-Spatial Working Memory and Individual Differences 2003, Psychology Press, Hove
20. Frigerio E, Burt D M, Gagliardi C, Cioffi G, Martelli S. Is everybody always my friend? Perception of approachability in Williams syndrome. Neuropsychologia 2006, 44:254-9.
21. Garner C, Callias M, Turk J. Executive function and theory of mind performance of boys with fragile-X syndrome. Journal of Intellectual Disability Research 1999, 43:466-74.
22. Gauthier L, Dehaut F, Joanette Y. The Bells Test: a quantitative and qualitative test for visual neglect. International Journal of Clinical Psychology 1989, 11:49-54.
23. Green C R, Mihic A M, Nikkel S M, Stade B C, Rasmussen C, Munoz D P. Executive function deficits in children with fetal alcohol spectrum disorders (FASD) measured using the Cambridge Neuropsychological Tests Automated Battery (CANTAB). Journal of Child Psychology and Psychiatry, and Allied Disciplines 2009, 50:688-97.
24. Helland T, Asbjørnsen A. Executive functions in dyslexia. Child Neuropsychoogy: a Journal on Normal and Abnormal Development in Childhood and Adolescence 2000, 6:37-48.
25. Hill E L. Executive dysfunction in autism. Cognitive Science 2004, 8:26-32.
26. Hooper S R, Hatton D, Sideris J, Sullivan K, Hammer J, Schaaf J. Executive functions in young males with fragile X syndrome in comparison to mental age-matched controls: baseline findings from a longitudinal study. Neuropsychology 2008, 22:36-47.
27. Jarrold C, Phillips C, Baddeley A D. Binding of visual and spatial short-term memory in Williams syndrome and moderate learning disability. Developmental Medicine and Child Neurology 2007, 49:270-3.
28. Jones W, Bellugi U, Lai Z, Chiles M, Reilly J, Lincoln A. II. Hypersociability in Williams Syndrome. Journal of Cognitive Neuroscience 2000, 12:30-46.
29. Kenworthy L E, Black D O, Wallace G L, Ahluvalia T, Wagner A E, Sirian L M. Disorganization: the forgotten executive dysfunction in high-functioning autism (HFA) spectrum disorders. Developmental Neuropsychology 2005, 28:809-27.
30. Kirk J W, Mazzocco M M, Kover S T. Assessing executive dysfunction in girls with fragile-or turner syndrome using the Contingency Naming Test (CNT). Developmental Neuropsychology 2005, 28:755-77.
31. Kittler P M, Krinsky-McHale S J, Devenny D A. Dual-task processing as a measure of executive function: a comparison between adults with Williams and Down syndromes. American Journal of Mental Retardation 2008, 113:117-32.
32. Korenberg J R, Chen X N, Hirota H, Lai Z, Bellugi U, Burian D. VI. Genome structure and cognitive map of Williams syndrome. Journal of Cognitive Neuroscience 2000, 12:89-107.
33. Lanfranchi S, Carretti B, Spanò G, Cornoldi C. A specific deficit in visuospatial simultaneous working memory in Down syndrome. Journal of Intellectual Disability Research 2009, 53:474-83.
34. Levin H S, Culhane K A, Hartmann J, Evankovich K, Mattson A J, Harward H. Developmental changes in performance on tests of purported frontal lobe functioning. Developmental Neuropsychology 1991, 7:377-95.
35. Lincoln A, Lai Z, Jones W. Shifting attention and joint attention dissociation in Williams syndrome: implications for the cerebellum and social deficits in autism. Neurocase: Cases Studies in Neuropsychology, Neuropsychiatry and Behavioral Neurology 2002, 8:226-32.
36. Lincoln A J, Searcy Y M, Jones W, Lord C. Social interaction behaviors discriminate young children with autism and Williams syndrome. Journal of the American Academy Child and Adolescent Psychiatry 2007, 46:323-31.
37. Manly T, Robertson I H, Anderson V, Nimmo-Smith I. The Test of Everyday Attention for Children (TEA-CH) 1999, Thames Valley Test Company, England, Bury St. Edmunds
38. Manly T, Anderson V, Nimmo-Smith I, Turner A, Watson P, Robertson I H. The differential assessment of Children's attention: the test of Everyday Attention for Children (TEA-Ch), normal sample and ADHD performance. Journal of Child Psychology and Psychiatry and Allied Disciplines 2001, 42:1065-81.
39. Mantyla T, Carelli M G, Forman H. Time monitoring and executive functioning in children and adults. Journal of Experimental Child Psychology 2007, 96:1-19.
40. Martens M A, Wilson S J, Reutens D C. Research review: Williams syndrome: a critical review of the cognitive, behavioral, and neuroanatomical phenotype. Journal of Child Psychology and Psychiatry 2008, 49:576-608.
41. Mervis C B, Klein-Tasman B P. Williams syndrome: cognition, personality, and adaptive behavior. Mental Retardation and Developmental Disabilities Research Reviews 2000, 6:148-58.
42. Mervis C B, Robinson B F, Bertrand J, Morris C A, Klein-Tasman B P, Armstrong S C. The Williams syndrome cognitive profile. Brain and Cognition 2000, 44:604-28.
43. Mervis C B, Morris C A, Klein-Tasman B P, Bertrand J, Kwitny S, Appelbaum L G. Attentional characteristics of infants and toddlers with Williams syndrome during triadic interactions. Developmental Neuropsychology 2003, 23:243-68.
44. Meyer-Lindenberg A, Mervis C B, Berman K F. Neural mechanisms in Williams syndrome: a unique window to genetic influences on cognition and behaviour. Nature Reviews Neuroscience 2006, 7:380-93.
45. Milner B, Corsi P, Leonard G. Frontal-lobe contribution to recency judgements. Neuropsychologia 1991, 29:601-18.
46. Miyake A, Shah P. Models of Working Memory: Mechanisms of Active Maintenance and Executive Control 1999, Cambridge University Press, New York
47. Miyake A, Friedman N P, Emerson M J, Witzki A H, Howerter A A, Wager T. The unity and diversity of executive functions and their contributions to complex 'frontal lobe' tasks: a latent variable analysis. Cognitive Psychology 2000, 41:49-100.
48. Mobbs D, Eckert M A, Mills D, Korenberg J, Bellugi U, Galaburda A M. Frontostriatal dysfunction during response inhibition in Williams syndrome. Biological Psychiatry 2006, 62:256-61.
49. Mobbs D, Eckert M A, Mills D, Korenberg J, Bellugi U, Galaburda A M. Frontostriatal dysfunction during response inhibition in Williams syndrome. Biological Psychiatry 2007, 62:256-61.
50. Nunnally J C, Bernstein I H. Psychometric Theory 1994, 3rd, edn., McGraw-Hill, New York
51. Oberauer K, Suss H M, Wilhelm O, Wittman W W. The multiple faces of working memory: storage, processing, supervision, and coordination. Intelligence 2003, 31:167-93.
52. O'Hearn K, Courtney S, Street W, Landau B. Working memory impairment in people with Williams syndrome: effects of delay, task and stimuli. Brain and Cognition 2009, 69:495-503.
53. Orsini A, Grossi D, Capitani E, Laiacona M, Papagno C, Vallar G. Verbal and spatial immediate memory span: normative data from 1355 adults and 1112 children. Italian Journal of Neurological Science 1987, 6:539-48.
54. Owen A M, Doyon J, Dagher A, Sadikot A, Evans A C. Abnormal basal ganglia outflow in Parkinson's disease identified with PET. Implications for higher cortical functions. Brain 1998, 121:949-65.
55. Pennington B F, Ozonoff S. Executive functions and developmental psychopathology. Journal of Child Psychology and Psychiatry 1996, 37:51-87.
56. Porter M A, Coltheart M, Langdon R. The neuropsychological basis of hypersociability in Williams and Down syndrome. Neuropsychologia 2007, 45:2839-49.
57. Rasmussen C. Executive functioning and working memory in fetal alcohol spectrum disorder. Alcoholism, Clinical and Experimental Research 2005, 29:1359-67.
58. Rhodes S M, Coghill D R, Matthews K. Methylphenidate restores visual memory, but not working memory function in attention deficit-hyperkinetic disorder. Psychopharmacology 2004, 175:319-30.
59. Rhodes S M, Coghill D R, Matthews K. Neuropsychological functioning in stimulant - naive boys with hyperkinetic disorder. Psychological Medicine 2005, 35:1109-20.
60. Rhodes S M, Riby D M, Park J, Fraser E, Campbell L E. Executive neuropsychological functioning in individuals with Williams syndrome. Neuropsychologia 2010, doi:10.1016/j.neuropsychologia.2009.12.021
61. Rinehart N J, Bradshaw J L, Moss S A, Brereton A V, Tonge B J. A deficit in shifting attention present in high-functioning autism but not Asperger's disorder. Autism: the International Journal of Research and Practice 2001, 5:67-80.
62. Roid G H, Miller L J. Leiter - R, Leiter International Performance Scale-Revised 2002, Giunti O.S. Organizzazioni Speciali, Firenze
63. Sampaio A, Sousa N, Férnandez M, Henriques M, Gonçalves O F. Memory abilities in Williams syndrome: dissociation or developmental delay hypothesis? Brain and Cognition 2008, 66:290-7.
64. Sannio Fancello G, Vio C, Cianchetti C. Torre di Londra. Test di Valutazione Delle Funzioni Esecutive (Pianificazione E Problem-Solving) 2006, Erikson, Trento
65. Scerif G, Cornish K, Wilding J, Driver J, Karmiloff-Smith A. Visual search in typically developing toddlers and toddlers with Fragile-or Williams syndrome. Developmental Science 2004, 7:116-30.
66. Sergeant J A, Geurts H, Oosterlaan J. How specific is a deficit of executive functioning for attention-deficit/hyperactivity disorder? Behavioural Brain Research 2002, 130:3-28.
67. Shallice T. Specific impairments of planning. Philosophical Transactions of the Royal Society of London 1982, 298:199-209.
68. Shallice T. From Neuropsychology to Mental Structure 1988, Cambridge University Press, Cambridge
69. Spinnler H, Tognoni G. Standardizzazione e taratura italiana di test neuropsicologici. Italian Journal of Neurological Sciences 1987, 8:35-7.
70. Strømme P, Bjornstad P G, Ramstad K. Prevalence estimation of Williams syndrome. Journal of Child Neurology 2002, 17:269-71.
71. Stroop J R. Studies of inference in serial verbal reactions. Journal of Experimental Psychology 1935, 18:643-62.
72. Tomc S A, Williamson N K, Pauli R M. Temperament in WS. American Journal of Medical Genetics 1990, 36:345-52.
73. Vicari S. PROMEA, Prove Di Memoria E Apprendimento Per L'età Evolutiva 2007, Giunti O.S. Organizzazioni Speciali, Firenze
74. Vicari S, Bellucci S, Carlesimo G A. Procedural learning deficit in children with Williams Syndrome. Neuropsychologia 2001, 39:665-77.
75. Vicari S, Bellucci S, Carlesimo G A. Visual and spatial working memory dissociation: evidence from Williams syndrome. Developmental Medicine and Child Neurology 2003, 45:269-73.
76. Vicari S, Bellucci S, Carlesimo G A. Evidence from two genetic syndromes for the independence of spatial and visual working memory. Developmental Medicine and Child Neurology 2006, 48:126-31.
77. Welsh M C. Developmental and clinical variations in executive functions. Developmental Variations in Learning 2002, 139-85. Molfese D LMolfese V J. In, eds, pp., Lawrence Erlbaum Associates, Inc, Mahwah, NJ
78. Welsh M C, Pennington B F, Groisser D B. A normative-developmental study of executive function: a window on prefrontal function in children. DevelopmentalNeuropsychology 1991, 7:131-49.