Frente a una concepción neuropsicológica estática, que entiende la arquitectura cognitiva como un conjunto de sistemas específicos de funcionamiento independiente de origen innato, este trabajo asume una perspectiva neu-roconstructivista, que contempla la especialización y localización progresiva de las funciones, enfatizando la necesidad de atender desde el desarrollo a los distintos niveles de explicación del funcionamiento psicológico, y en el que la relación con las bases genéticas o neurobiológicas también va a estar mediada por un ambiente dinámico. Desde estos argumentos se revisa la evidencia experimental sobre el funcionamiento de las personas con síndrome de Williams (SW) en distintos aspectos vinculados con la cognición social (competencias mentalistas, procesamiento de rostros y lenguaje). Se concluye en contra de la propuesta de que el perfil psicológico de las personas con SW evidencia la existencia de un módulo para la cognición social, se sugiere la necesidad de atender a las trayectorias de desarrollo y se plantean algunas implicaciones del modelo para la intervención psicológica.
Get full access to this article
View all access options for this article.
References
1.
AnnazD.Karmiloff-SmithA.JohnsonM.ThomasM. (2009). A cross-syndrome study of the development of holistic face recognition in children with autism, Down syndrome, and Williams syndrome. Journal of Experimental Child Psychology, 102, 456–486. doi: 10.1016/j.jecp.2008.11.005
2.
BellugiU.LichtenbergerL.JonesWLaiZ.St. GeorgeM. (2000). The neurocognitive profile of Williams syndrome: A complex pattern of strengths and weakness. Journal of Cognitive Neuroscience, 12, 7–29. doi: 10.1162/089892900561959
3.
BellugiU.SaboH.VaidJ. (1988). Spatial deficits in children with Williams Syndrome. EnStiles-DavisJ.KritchevskyM. (Eds.), Spatial cognition: Brain bases and development (pp. 273–298). Hillsdale, NJ: Lawrence Erlbaum Associates.
4.
BellugiU.WangP.JerniganT. (1994). Williams Syndrome: an unusual neuropsychological profile. EnBromanS. H.GrafmanJ. (Eds.), Atypical cognitive deficits in developmental disorders (pp. 23–56). Hillsdale, Nueva York: Erlbaum.
5.
BrothersL.RingB. (1992). A neuroethological framework for the representation of minds. Journal of cognitive neuroscience, 4, 107–118. doi: 10.1162/jocn.1992.4.2.107
6.
BrownJ.JohnsonM.PatersonS.GilmoreR.GsödlM.LonghiE.Karmiloff-SmithA. (2003). Spatial representation and attention in toddlers with Williams syndrome and Down syndrome. Neuropsychologia, 41, 1037–1046. doi: 10.1016/S0028-3932(02)00299-3
7.
CamposR. (2009). Construyendo mentes: Desarrollo de la comprensión de estados mentales en niños con síndrome de Williams y en desarrollo típico. Tesis doctoral no publicada, Universidad Autónoma de Madrid.
8.
CamposR.SotilloM. (2012). Mental state understanding and social interaction. EnFarranE. K.Karmiloff-SmithA. (Eds.), Neurodevelopmenal disorders across the lifespan (pp. 279–295). Nueva York: Oxford University Press.
9.
DaiL.BellugiU.ChenX.Pulst- KorenbergA.Jarvinen-PasleyA.Tirosh-WagnerT.EisP.GrahamJ.MillsD.SearcyY.KorenbergJ. (2009). Is it Williams syndrome? GTF2IRD1 implicated in visual-spatial construction and GTF2I in sociability revealed by high resolution arrays. American Journal of Medical Genetics A, 149A, 302–314. doi: 10.1002/ajmg.a.32652
10.
FidlerD.HepburnS.MostD.PhilofskyA.RogersS. (2007). Emotional responsivity in young children with Williams syndrome. American Journal on Mental Retardation, 112, 194–206. doi: 10.1038/ng0993-11
11.
GagliardiC.FrigerioE.BurtD. M.CazzanigaI.PerrettD. I.BorgattiR. (2003). Facial expression recognition in Williams syndrome. Neuropsychologia, 41, 733–738. doi: 10.1016/S0028-3932(02)00178-1
12.
GolaraiG.HongS.HaasB.GalaburdaA.MillsD.BellugiU.ReissA. (2010). The fusiform face area is enlarged in Williams syndrome. Journal of Neuroscience, 30, 6700–6712. doi: 10.1523/JNEUR0SCI.4268-09.2010
13.
GoschA.StandingG.PankauR. (1994). Linguistic abilities in children with Williams-Beuren syndrome. American Journal of Medical Genetics, 52, 291–296. doi: 10.1002/ajmg.1320520308
14.
GriceS.SpratlingM.Karmiloff-SmithA.HalitH.CsibraG.De-HaanM.JohnsonM. (2001). Disordered visual processing and oscillatory brain activity in autism and Williams syndrome. Neuroreport, 12, 2697–2700. doi: 10.1097/00001756-200108280-00021
15.
HaasB.HoeftF.SearcyY.MillsD.BellugiU.ReissA. (2010). Individual differences in social behavior predict amygdala response to fearful facial expressions in Williams syndrome. Neuropsychologia, 48, 1283–1288. doi: 10.1016/j.neuropsychologia.2009.12.030
16.
HaasB.MillsD.YamA.HoeftF.BellugiU.ReissA. (2009). Genetic influences on sociability: Heightened amygdala reactivity and event-related responses to positive social stimuli in Williams syndrome. Journal of Neuroscience, 29, 1132–1139. doi: 10.1523/JNEUR0SCI.5324-08.2009
17.
HowlinP.DaviesM.UdwinO. (1998). Cognitive functioning in adults with Williams syndrome. Journal of Child Psychology and Psychiatry, 39, 183–189. doi: 10.1111/1469-7610.00312
18.
JohnA.RoweM.MervisC. (2009). Referential communication skills of children with Williams syndrome: Understanding when messages are not adequate. American Journal on Intellectual and Developmental Disabilities, 114, 85–99. doi: 10.1352/2009.114.85-99
19.
JohnsonS.CareyS. (1998). Knowledge enrichment and conceptual change in folk biology: Evidence from Williams syndrome. Cognitive Psychology, 37, 156–200. doi: 10.1006/cogp.1998.0695
20.
JonesW.BellugiU.LaiZ.ChilesM.ReillyJ.LincolnA.AdolphsR. (2000). Hypersociability in Williams syndrome. Journal of Cognitive Neuroscience, 12, 30–46. doi: 10.1162/089892900561968
21.
Karmiloff-SmithA. (1997). Crucial differences between developmental cognitive neuroscience and adult neuropsychology. Developmental Neuropsychology, 13, 513–524. doi: 10.1080/87565649709540693
22.
Karmiloff-SmithA. (1998). Developmental itself is the key to understanding developmental disorders. Trends in Cognitive Sciences, 2, 389–398. doi: 10.1016/S1364-6613(98)01230-3
Karmiloff-SmithA. (2012). Brain: The neuroconstructivist approach. EnFarranE. K.Karmiloff-SmithA., Neurodevelopmenal disorders across the lifespan (pp. 37–58). Nueva York: Oxford University Press.
25.
Karmiloff-SmithA.BroadbentH.FarranE.LonghiE.D'souzaD.MetcalfeK.TassabehjiM.WuR.SenjuA.HappéF.TurnpennyP.SansburyF. (2012). Social cognition in Williams syndrome: Genotype/phenotype insights from partial deletion patients. Frontiers in Psychology, 3, 168. doi: 10.3389/fpsyg.2012.00168
26.
Karmiloff-SmithA.GrantJ.BerthoudI.DaviesM.HowlinP.UdwinO. (1997). Language and Williams syndrome: How intact is “intact”?Child Development, 68, 246–262. doi: 10.1111/j.14678624.1997.tb01938.x
27.
Karmiloff-SmithA.KlimaE.BellugiU.GrantJ.Baron-CohenS. (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
28.
Karmiloff-SmithA.ThomasM. (2003). What can developmental disorders tell us about the neurocomputational constraints that shape development? The case of Williams syndrome. Development and Psychopathology, 15, 969–990. doi: 10.1017/S0954579403000476
29.
Karmiloff-SmithA.ThomasM.AnnazD.HumphreysK.EwingS.BraceN.DuurenM.PikeG.GriceS.CampbellR. (2004). Exploring the Williams syndrome face processing debate: The importance of building developmental trajectories. Journal of Child Psychology and Psychiatry, 45, 1258–1274. doi: 10.1111/j.1469-7610.2004.00322.x
30.
Klein-TasmanB.MervisC.LordC.PhillipsK. (2007). Socio-communicative deficits in young children with Williams syndrome: Performance on the Autism Diagnostic Observation Schedule. Child Neuropsychology, 13, 444–467. doi: 10.1080/09297040601033680
31.
LaingE.ButterworthG.AnsariD.GsödlM.LonghiE.PanagiotakiG.PatersonS.Karmiloff-SmithA. (2002). Atypical development of language and social communication in toddlers with Williams syndrome. Developmental Science, 5, 233–246. doi: 10.1111/1467-7687.00225
32.
LaingE.JarroldC. (2007). Comprehension of spatial language in Williams syndrome: Evidence for impaired spatial representation of verbal descriptions. Clinical Linguistics and Phonetics, 21, 689–704. doi: 10.1080/02699200701541441
33.
LawsG.BishopD. (2004). Pragmatic language impairment and social deficits in Williams syndrome: A comparison with Down's syndrome and specific language impairment. International Journal of Language and Communication Disorders, 39, 45–64. doi: 10.1080/13682820310001615797
34.
LevitinD.ColeK.LincolnA.BellugiU. (2005). Aversion, awareness, and attraction: Investigating claims of hyperacusis in the Williams syndrome phenotype. Journal of Child Psychology and Psychiatry, 46, 514–523. doi: 10.1111/j.1469-7610.2004.00376.x
35.
MalenfantP.LiuX.HudsonM.QiaoY.HrynchakM.RiendeauN.HildebrandM.CohenI.ChudleyA.Forster-GibsonC.MickelsonE.Rajcan-SeparovicE.LewisM.HoldenJ. (2011). Association of GTF2i in the Williams-Beuren syndrome critical region with autism spectrum disorders. Journal of Autism and Developmental Disorders, 42, 1459–69. doi: 10.1007/s10803-011-1389-4
36.
MareschalD.JohnsonM.SiroisS.SpratlingM.ThomasM.WestermannG. (2007). Neuroconstructivism, Vol. I: How the brain constructs cognition.Oxford: Oxford University Press.
37.
MartensM.WilsonS.DudgeonP.ReutensD. (2009). Approachability and the amygdala: Insights from Williams syndrome. Neuropsychologia, 47, 2446–2453. doi: 10.1016/j.neuropsychologia.2009.04.017
38.
Martínez-castillaP.SotilloM.CamposR. (2011). Prosodic abilities of Spanish-speaking adolescents and adults with Williams syndrome. Language and Cognitive Processes, 26, 1055–1082. doi: 10.1080/01690965.2010.504058
39.
MervisC.BertrandJ. (1997). Developmental Relations between Cognition and Language: Evidence from Williams Syndrome. EnAdamsonL. B.RomskyM. A. (Eds.), Communication and Language Acquisition: Discoveries from Atypical Development (pp. 75–160). Baltimore: Paul Brookes Publishing.
40.
MervisC.MorrisC.BertrandJ.RobinsonF. R. (1999). Williams Syndrome: findings from an integrated program of research. EnTager-FlusbergH. (Ed.), Neurodevelopmental disorders: contribution to a new framework from the cognitive neurosciences (pp. 665–110). Cambridge, MA: MIT Press.
41.
MervisC.MorrisC.Klein-TasmanB.BertrandJ.KwitnyS.AppelbaumL.RiceC. (2003). Attentional characteristics of infants and toddlers with Williams syndrome during triadic interactions. Developmental Neuropsychology, 23, 243–68. doi: 10.1080/87565641.2003.9651894
42.
MillsD.AlvarezT.GeorgeM.ApplebaumL.BellugiU.NevilleH. (2000). Electrophysiological studies of face processing in Williams syndrome. Journal of Cognitive Neuroscience, 12, 47–64. doi: 10.1162/089892900561977
Plesa-SkwererD.FajaS.SchofieldC.VerbalisA.Tager-FlusbergH. (2006a). Perceiving facial and vocal expressions of emotion in individuals with Williams syndrome. American Journal on Mental Retardation, 111, 15–26. doi: 10.1352/0895-8017(2006)111[15:PFAVE0]2.0.C0;2)
45.
Plesa-SkwererD.VerbalisA.SchofieldC.FajaS.Tager-FlusbergH. (2006b). Social-perceptual abilities in adolescents and adults with Williams syndrome. Cognitive Neuropsychology, 23, 338–349. doi: 10.1080/02643290542000076
46.
PorterM.ColtheartM.LangdonR. (2007). The neuropsychological basis of hypersociability in Williams and Down syndrome. Neuropsychologia, 45, 2839–2849. doi: 10.1016/j.neuropsychologia.2007.05.006
47.
PorterM.ColtheartM.LangdonR. (2008). Theory of mind in Williams Syndrome assessed using a nonverbal task. Journal of Autism and Developmental Disorders, 38, 806–814. doi: 10.1007/s1083-007-0447-4
48.
ReillyJ.KlimaE.BellugiU. (1990). Once more with feeling: Affect and language in atypical populations. Development and Psychopathology, 2, 367–391. doi: 10.1017/S0954579400005782
49.
RibyD.Doherty-SneddonG.BruceV. (2008). Atypical unfamiliar face processing in Williams syndrome: What can it tell us about typical familiarity effects?Cognitive Neuropsychiatry, 13, 47–58. doi: 10.1080/13546800701779206
50.
RibyD.HacockP. (2009). Do faces capture attention of individuals with Williams syndrome or autism? Evidence from tracking eye movements. Journal of Autism and Developmental Disorders, 39, 421–431. doi: 10.1007/s10803-008-0641-z
51.
RossenM.JonesW.WangP.KlimaE. (1995). Face processing: Remarkable sparing in Williams syndrome. Genetic Counseling, 6, 138–140.
52.
SotilloM.García NogalesM. A.CamposR. (2007). Teoría de la mente y lenguaje: El caso del síndrome de Williams. Infancia y Aprendizaje, 30, 459–74. doi: 10.1174/021037007781787534
53.
StojanovikV. (2006). Social interaction deficits and conversational inadequacy in Williams syndrome. Journal of Neurolinguistics, 19, 157–173. doi: 10.1016/j.jneuroling.2005.11.005
54.
StojanovikV.PerkinsM.HowardS. (2004). Williams syndrome and specific language impairment do not support claims for developmental double dissociations and innate modularity. Journal of Neurolinguistics, 17, 403–424. doi: 10.1016/j.jneuroling.2004.01.002
55.
SullivanK.Tager-FlusbergH. (1999). Second-order belief attribution in Williams syndrome: intact or impaired?American Journal on Mental Retardation, 104, 523–532. doi: 10.1352/0895017(1999)104<0523:SBAIWS>2.0.CG;2
56.
Tager-FlusbergH.SullivanK. (2000). A componential view of theory of mind: Evidence from Williams syndrome. Cognition, 16, 59–89. doi: 10.1016/S0010-0277(00)00069-X
57.
Tager-FlusbergH.SullivanK.BoshartJ. (1997). Executive functions and performance on false belief tasks. Developmental Neuropsychology, 13, 487–493. doi: 10.1080/87565649709540689
58.
ThomasM.Van DuurenM.PurserH.MareschalD.AnsariD.Karmiloff-SmithA. (2010). The development of metaphorical language comprehension in typical development and in Williams syndrome. Journal of Experimental Child Psychology, 106, 99–224. doi: 10.1016/j.jecp.2009.12.007
59.
VolterraV.CapirciO.PezziniG.SabbadiniL.VicariS. (1996). Linguistic abilities in Italian children with Williams syndrome. Cortex, 32, 663–677.
60.
YoungE.LipinaT.TamE.MandelA.ClapcoteS.BechardA.ChambersJMountH.FletcherP.RoderJ.OsborneL. (2008). Reduced fear and aggression and altered serotonin metabolism in Gtf2ird1-targeted mice. Genes Brain and Behavior, 7, 224–234. doi: 10.1111/j.1601-183X.2007.00343.x
