BOUNCE koglist(a)www.cogpsyphy.hu: Header field too long (>1024) From: "Csaba Pleh" <pleh(a)itm.bme.hu> To: "Vidnyanszky Zoltan" <vidnyanszky(a)ana.sote.hu>hu>, "Vajda Zsuzsanna" <vajda.zsuzsanna(a)drotposta.hu>hu>, "Vajda Zsuzsanna" <vajdazs(a)edpsy.u-szeged.hu>hu>, =?iso-8859-1?Q?T=E1rnok_Zsanett?= <tarnok(a)vadaskertalapitvany.hu>hu>, "szendi" <szendi(a)nepsy.szote.u-szeged.hu>hu>, "Sandor Klara" <sandor(a)jgytf.u-szeged.hu>hu>, "Peter Molnar" <pmolnar(a)jaguar.dote.hu>hu>, =?iso-8859-1?Q?P=E9ley_Bernadette?= <peley(a)btk.pte.hu>hu>, =?iso-8859-1?B?UGFwIErhbm9z?= <eurania(a)mail.uti.hu>hu>, "Pleh" <pleh(a)itm.bme.hu>hu>, "Santha Miklos" <Santha(a)nucleus.szbk.u-szeged.hu>hu>, "Kri Szabolcs" <SZKERI(a)phys.szote.u-szeged.hu>hu>, "Janka Zoltan professzor" <janka(a)nepsy.szote.u-szeged.hu>hu>, "Beke Monika" <b.moni(a)itm.bme.hu>hu>, "gadoros" <gadoros(a)axelero.hu>hu>, "Gyori" <GyoriMiklos(a)axelero.hu>hu>, "halasz" <halasz(a)opni.hu>hu>, "Kovacs Gyula" <g.kovacs(a)richter.hu>hu>, "Kovacs Ilona" <ikovacs(a)cyclops.rutgers.edu>du>, "Lukacs" <alukacs(a)axelero.hu>hu>, "Racsmany" <racsmany(a)edpsy.u-szeged.hu>hu>, "Laszlo Janos" <laszlo(a)mtapi.hu>hu>, =?iso-8859-1?B?S2924WNzIEtyaXN082Y=?= <kristof.k(a)chello.hu>hu>, "Krajcsi Attila" <krajcsi(a)izabell.elte.hu>hu>, "Korossy Judit" <korossy(a)edpsy.u-szeged.hu>hu>, =?iso-8859-1?B?S/NueWEgQW5pa/M=?= <konya(a)izabell.elte.hu>hu>, =?iso-8859-1?Q?Ko=F3s_Orsolya?= <kooso(a)mtapi.hu>hu>, "Kenesei Istvan" <kenesei(a)sol.cc.u-szeged.hu>hu>, "Hamori Eszter" <ehamori(a)mtapi.hu>hu>, =?iso-8859-1?Q?Gergely_Gy=F6rgy?= <gergelyg(a)mtapi.hu>hu>, "Futo Judit" <tokimano(a)yahoo.com>om>, =?iso-8859-1?B?RmVo6XJN?= <feherm(a)phil.philos.bme.hu>hu>, =?iso-8859-1?B?RfZycyBTemF0aG3hcnk=?= <szathmary(a)colbud.hu>hu>, "Dr. Pogany Gabor" <pogany(a)williams.ngo.hu>hu>, =?iso-8859-1?Q?Dank=F3_Bertalan?= <danko.bertalan(a)axelero.hu>hu>, <Csilla.Gyenis(a)colbud.hu>hu>, =?iso-8859-1?Q?Csibra_Gerg=F5?= <g.csibra(a)bbk.ac.uk>uk>, =?iso-8859-1?Q?Cs=E1nyi_Vilmos?= <csanyi13(a)axelero.hu>hu>, =?iso-8859-1?Q?Czigler_Istv=E1n?= <czigler(a)cogpsyphy.hu>hu>, "Boross Ottilia" <ottilia(a)btk.ppke.hu>hu>, "Barotfi Edit" <barotfi(a)zeus.colbud.hu>hu>, "Balazs Gulyas" <Balazs.Gulyas(a)neuro.ki.se>se>, "Aurel GABRIS" <gabrisa(a)optics.szfki.kfki.hu>hu>, "Attila Krajcsi" <krajcsi(a)edpsy.u-szeged.hu>hu>, "Agnes Kovacs" <agikov(a)hotmail.com>om>, =?iso-8859-1?B?wWThbSBHefZyZ3k=?= <dradam(a)ludens.elte.hu>hu>, "koglist" <koglist(a)cogpsyphy.hu>hu>, <ajuska(a)axelero.hu>hu>, =?iso-8859-1?Q?Andr=E1s_Rung?= <rungandras(a)hotmail.com>om>, "Gabi Felhosi" <felhogab(a)hotmail.com>om>, "Gabor Zemplen" <zemplen(a)hps.elte.hu>hu>, "Gabris Krisztian" <gabrikri(a)freemail.hu>hu>, "Gervain Judit" <h633301(a)stud.u-szeged.hu>hu>, "Lilla" <mlillus(a)izabell.elte.hu>hu>, "Mund Katalin" <mundka(a)freemail.hu>hu>, "noemi" <hahnoemi(a)freemail.hu> Subject: Ursula Bellugi es A. Karmiloff -Smith eloadasa majus 2-an a Collegium Budapestben Date: Tue, 23 Apr 2002 12:46:12 +0200 --- The Rector and Permanent Fellows of COLLEGIUM BUDAPEST / Institute for Advanced Study invite you to a Public Session by Ursula Bellugi and Annette Karmiloff-Smith on Thursday, 2 May 2002, at 5.00 p.m. in Collegium Budapest 1014 Budapest Szentháromság u. 2. The session is organized on the occasion of the workshop held at the Collegium on METHODOLOGICAL ASPECTS OF WILLIAMS SYNDROME RESEARCH organized by the Collegium and BME Center for Cognitive Science Linking Cognitive Neuroscience and Molecular Genetics: New Perspectives from Williams Syndrome Ursula Bellugi Edward S. Klima Laboratory for Cognitive Neuroscience The Salk Institute for Biological Studies La Jolla, CA Introduction to Williams Syndrome. Recent major advances in cognitive neuroscience, in brain imaging and molecular genetics make this an ideal time to begin to examine the links between these levels through the study of specific genetic syndromes. We present an historical approach which contrasts different genetically based disorders such as Williams syndrome with others such as Down syndrome. Williams syndrome is a rare genetically-based disorder resulting in a fascinating and puzzling profile of peaks and valleys of abilities. Starting at a point in time where little to nothing was known about the syndrome, we will show how initial studies have found that the syndrome results in specific dissociations in higher cognitive functions, both within and across domains: a) cognitive deficits but relative strength in language; b) extreme disorders in spatial cognition but strength in face processing; as well as c) hypersociability. Studies of cognitive functions, brain structure, brain function, and molecular genetics resulting from such syndromes can thus help begin to forge links between disparate levels, including brain, gene, and cognition. Neurobiological Findings Contrasting Genetic Syndromes. We will present initial findings from studies of Williams syndrome involving cognitive, neuromorphological, neurophysiological, brain cytoarchitectonic and genetic probes. The neuromorphological characterization of Williams syndrome using quantitative high resolution voluming of magnetic resonance images (MRI) suggests that there are differential areas of brain development in two contrasting genetic syndromes. Although cerebral volumes are smaller than normal in both Williams syndrome and Down syndrome subjects, anterior brain volume and limbic structure volume appear proportionately better preserved in Williams syndrome, and neocerebellar volumes are actually enlarged. Primary auditory areas also appear to be disproportionately large in Williams syndrome subjects, compared to Down syndrome and normal controls, perhaps subserving relative strengths in language and affinity for music. We will present results of initial studies in language, face, and space processing that provide clues to neurophysiological phenotypic markers in Williams syndrome. Summary: These studies may in the long run provide new clues to the neurobiological basis of domains of higher cognitive functioning. The finding that specific brain regions are selectively preserved and impaired in diverse genetically-based syndromes suggests that they may come under the influence of specific genetic developmental factors, or that their development is mutually interactive. Such issues linking cognitive phenotypes, neural systems and molecular genetics may have implications for cortical plasticity, and are important to central questions of cognitive neuroscience. CV of Dr. Ursula Bellugi: she is Professor at The Salk Institute for Biological Studies, as well as Director of the Laboratory for Cognitive Neuroscience, and Adjunct Professor at UCSD and SDSU, and an Associate with the Sloan Center for Theoretical Neurobiology. Much of her research is in collaboration with her husband, Edward S. Klima. Both have doctorates from Harvard University and they have received many awards for their research, including two MERIT awards from the National Institute of Child Health and Development, a Neuroscience Investigator Award from the National Institute on Deafness., and the Distinguished Scientific Contribution Award from the American Psychological Association. Dr. Bellugi has also received the Foundation IPSEN Prize in Neuronal Plasticity, together with Torsten Wiesel and Wolfgang Singer. Bellugi and Klima's research is focused on the biological foundations of language and other cognitive functions and their genetic basis. Bellugi and Klima have co-authored several hundred papers and books including The Signs of Language and What the Hands Reveal About the Brain as well as the recent book Journey from Cognition to Brain to Gene: Perspectives from Williams Syndrome (which has been nominated for an award). These studies permit the exploration of some of the central issues of cognitive neuroscience linking cognitive functions with their bases in neurobiology and gene expression. Genotype Phenotype Relations: Why a Cognitive Developmental Perspective is Crucial Annette Karmiloff-Smith I will discuss three reasons why genotype/phenotype mapping is not straight-forward even in a genetic disorder where the deleted genes and the pattern of behavioural impairments have already been identified. The case of Williams syndrome (WS) serves as a model. First, genotype/phenotype relations will be discussed with respect to older children and adults with WS, compared to non-WS individuals with similar but smaller deletions on chromosome 7q11.23. Our studies suggest that it is at the level of underlying *cognitive* processes, and not behavioural outcomes, that genotype/phenotype relations must be explored. Second, I will show how even in domains where people with WS score in the range of normal controls, the cognitive processes they use are different. Third, I will argue that it essential to consider genotype/phenotype relations in early infancy and not solely in the phenotypic outcome. The infant brain is not like a Swiss army knife, simply handed down by evolution with preformed, specialized components that may be individually impaired at birth. Rather, subtle impairments early on can have a differential impact on different domains in the phenotypic outcome. Ontogenetic development itself is the clue to understanding developmental disorders and their relation to the structure of the adult cognitive system. CV of Annette Karmiloff-Smith: she Professor and Head of the Neurocognitive Development Unit at the Institute of Child Health in London where she runs a research team examining cognitive development in genetic disorders. She has a Doctorat en Psychologie Génétique et Expérimentale from the University of Geneva, where she studied and worked with the Swiss psychologist, Jean Piaget, at the Centre International d'Epistemologie Genetique. She is a Fellow of the British Academy and of the Academy * of Medical Sciences. In 1995, she was awarded the British Psychological Society's Book Award for excellence in the literature of psychology for her book Beyond Modularity: A developmental perspective on cognitive science (MIT Press, 1992). Her co-authored book, Rethinking Innateness: A Connectionist Perspective on Development, (MIT Press, 1996) was nominated for the 1997 American Psychological Association Eleanor Maccoby Prize. With her daughter, Kyra Karmiloff, she has written two books bringing science to the general public. Author of 7 books and of some 200 chapters and articles in scientific journals, her works have been translated into Japanese, Chinese, Italian, Spanish, French, Greek, Hungarian, Arabic and Hebrew. Csaba Pleh, Professor of Psychology Budapest U. of Technology and Economics, Center for Cognitive Science Presently at Collegium Budapest, Budapest, Szentharomsag u 2 H-1014 cspleh@ colbud.hu, T: 3612248323, Fax: 3612248310 Mobile: (06)303500431