Dethroning the Myth: Cognitive Dissociations and Innate Modularity in Williams Syndrome

Despite increasing empirical data to the contrary, it continues to be claimed that morphosyntax and face processing skills of people with Williams syndrome are intact. This purported intactness, which coexists with mental retardation, is used to bolster claims about innately specified, independently functioning modules, as if the atypically developing brain were simply a normal brain with parts intact and parts impaired. Yet this is highly unlikely, given the dynamics of brain development and the fact that in a genetic microdeletion syndrome the brain is developing differently from the moment of conception, throughout embryogenesis, and during postnatal brain growth. In this article, we challenge the intactness assumptions, using evidence from a wide variety of studies of toddlers, children, and adults with Williams syndrome.

[1]  Carolyn B. Mervis,et al.  COGNITIVE AND BEHAVIORAL GENETICS '99 Visuospatial Construction , 1999 .

[2]  A D Baddeley,et al.  The Children's Test of Nonword Repetition: a test of phonological working memory. , 1994, Memory.

[3]  Virginia Volterra,et al.  Linguistic Abilities in Italian Children With Williams Syndrome , 1996, Cortex.

[4]  Attentional Characteristics of Infants and Toddlers With Williams Syndrome During Triadic Interactions , 2003, Developmental neuropsychology.

[5]  U Bellugi,et al.  Cerebral morphologic distinctions between Williams and Down syndromes. , 1993, Archives of neurology.

[6]  Steven Pinker,et al.  Words and rules , 1998 .

[7]  Virginia Valian,et al.  A study of relative clauses in Williams syndrome , 2002, Journal of Child Language.

[8]  U Bellugi,et al.  Callosal morphology concurs with neurobehavioral and neuropathological findings in two neurodevelopmental disorders. , 1992, Archives of neurology.

[9]  David I. Perrett,et al.  Recognition of individual faces and average face prototypes by 1- and 3-month-old infants , 2001 .

[10]  Carolyn B. Mervis,et al.  Contrasting Patterns of Cognitive Abilities of 9- and 10-Year-Olds With Williams Syndrome or Down Syndrome , 1999 .

[11]  Peter La Cour,et al.  Rivermead Behavioural Memory Test , 1990 .

[12]  Susan Carey,et al.  Knowledge Enrichment and Conceptual Change in Folkbiology: Evidence from Williams Syndrome , 1998, Cognitive Psychology.

[13]  Ursula Bellugi,et al.  Seeing either the forest or the trees: Dissociation in visuospatial processing , 1989, Brain and Cognition.

[14]  G. Carlesimo,et al.  Short-term memory in children with Williams syndrome: A reduced contribution of lexical-semantic knowledge to word span , 1996, Neuropsychologia.

[15]  Julia Grant,et al.  Do Individuals with Williams Syndrome have Bizarre Semantics? Evidence for Lexical Organization Using an On-Line Task , 1997, Cortex.

[16]  M Davies,et al.  Language and Williams syndrome: how intact is "intact"? , 1997, Child development.

[17]  Ursula Bellugi,et al.  Contrasting profiles of language development in children with williams and down syndromes , 1997 .

[18]  Ursula Bellugi,et al.  I. The Neurocognitive Profile of Williams Syndrome: A Complex Pattern of Strengths and Weaknesses , 2000, Journal of Cognitive Neuroscience.

[19]  Harald Clahsen,et al.  Syntax and morphology in Williams syndrome , 1998, Cognition.

[20]  Morris Halle,et al.  The rules of language , 1980, IEEE Transactions on Professional Communication.

[21]  C. Mervis,et al.  Global Spatial Organization by Individuals with Williams Syndrome , 1999 .

[22]  Lawrence G. Appelbaum,et al.  III. Electrophysiological Studies of Face Processing in Williams Syndrome , 2000, Journal of Cognitive Neuroscience.

[23]  A. Karmiloff-Smith,et al.  Cognitive modularity and genetic disorders. , 1999, Science.

[24]  J. Greene Contributions to Neuropsychological Assessment , 1995 .

[25]  A. Karmiloff-Smith Development itself is the key to understanding developmental disorders , 1998, Trends in Cognitive Sciences.

[26]  W. Yule,et al.  A cognitive and behavioural phenotype in Williams syndrome. , 1991, Journal of clinical and experimental neuropsychology.

[27]  Carolyn B. Mervis,et al.  Williams syndrome: Findings from an integrated program of research , 1999 .

[28]  Brown Jh,et al.  The development of visual cognition in infants with Williams and Down's syndromes. , 2000 .

[29]  Laura Lakusta,et al.  Past tense formation in Williams syndrome , 2001 .

[30]  S. Pinker The Language Instinct , 1994 .

[31]  Linda Chang,et al.  Brain biochemistry in Williams syndrome: evidence for a role of the cerebellum in cognition? , 1999, Neurology.

[32]  Alan M. Leslie,et al.  Pretense, Autism, and the Theory-of-Mind Module , 1992 .

[33]  A. Karmiloff-Smith,et al.  Early Word Segmentation by Infants and Toddlers With Williams Syndrome , 2003 .

[34]  N. Bayley Bayley Scales of Infant Development , 1999 .

[35]  Terry L. Jernigan,et al.  Williams syndrome: An unusual neuropsychological profile. , 1994 .

[36]  U. Bellugi,et al.  Neuropsychological, neurological, and neuroanatomical profile of Williams syndrome. , 2005, American journal of medical genetics. Supplement.

[37]  A. Karmiloff-Smith,et al.  Phonological Short-term Memory and its Relationship to Language in Williams Syndrome. , 1997, Cognitive neuropsychiatry.

[38]  C. Deruelle,et al.  Configural and Local Processing of Faces in Children with Williams Syndrome , 1999, Brain and Cognition.

[39]  Mark H Johnson,et al.  Spatial representation and attention in toddlers with Williams syndrome and Down syndrome , 2003, Neuropsychologia.

[40]  A. Karmiloff-Smith,et al.  Learning to read in Williams syndrome: looking beneath the surface of atypical reading development. , 2001, Journal of child psychology and psychiatry, and allied disciplines.

[41]  Michael W. Spratling,et al.  Disordered visual processing and oscillatory brain activity in autism and Williams Syndrome , 2001, Neuroreport.

[42]  Constructivism, nativism, and explanatory adequacy , 1997, Behavioral and Brain Sciences.

[43]  S. de Schonen,et al.  Hemispheric asymmetry in a face discrimination task in infants. , 1990, Child development.

[44]  C. Temple Cognitive neuropsychology and its application to children. , 1997, Journal of child psychology and psychiatry, and allied disciplines.

[45]  M. Ullman,et al.  Past tense morphology in specifically language impaired and normally developing children , 2001 .

[46]  O. Braddick,et al.  A specific deficit of dorsal stream function in Williams' syndrome , 1997, Neuroreport.

[47]  Ursula Bellugi,et al.  Bridging cognition, the brain and molecular genetics: evidence from Williams syndrome , 1999, Trends in Neurosciences.