May 1.		ARRIVALS
	17:20	We will take you to the boat landing from the Guesthouse
	18:00-20:00	River-boat excursion in the afternoon (Boat is named Hófehérke, Snow-white)
	20:00-	Dinner at Dunaparti Vedégl?, Halász utca 1.
May 2.		MODELS AND DATA
	9:00-13:00	Morning session: Language profiles in WS (Lukács, Ackerman, Volterra, Thomas)
	14:00-16:00	Afternoon session: Sociality: WS and autism (Gy?ri, Káldy)
	17:00-19:00	Public session (evening): Determinants of development in WS (Bellugi, Karmiloff-Smith)
	20:00-	Dinner in small groups
May 3.		MODELS AND DATA
	9:00-13:00	Morning session: WS and working memory (Jarrold, Racsmány, Vicari)
	14:00-17:00	Afternoon session: Visual organization in WS (Atkinson, Kovács, Jariabková)
	17:00-	Occasional sight seeing
	19:00-	Opera for those who signed up
May 4.		INTERACTIONS
	9:00-11:00	Interactions between parents and scientists (Pogány)
	13:00-17:30	Afternoon session: Methods Vision Memory and neuropsychology (Jarrold,)
	19:00-	Evening party at Csaba Pléh
May 5.		Free program

Detailed scientific program

May 2.		MODELS AND DATA
	9:00-13:00	Morning session: Language profiles in WS (Volterra, Lukács, Thomas, Ackerman)
	9:00-9:45	Virginia Volterra Early linguistic abilities in Italian children with WS
	9:45-10:30	Ágnes Lukács: Frequency effects in the WS mental lexicon
	10:30-11:00	Coffee break
	11:00-11:45	Michael Thomas: Computational modeling of language acquisition in Williams syndrome: The case of past tense formation
	11:45-12.30	Farrell Ackerman: Rules and WS general discussion on language
	12:30-14.00
	14:00-16:00	Afternoon session: Sociality: WS and autism (Gy?ri, Káldy)
	14:00-14:45	Miklós Gy?ri:Fractionating 'theory of mind': evidence from autism
	14:45-15:30	Zsuzsa Káldy: Discussion on sociality and WS
	15:30-16.00
	16.00-16.30	Coffee break
	17:00-19:00	Public session: Determinants of development in WS (Beluggi, Karmiloff-Smith)
	17:00-18:00	Ursula. Bellugi & Edward. Klima: Linking Cognitive Neuroscience and Molecular Genetics: New Perspectives from Williams Syndrome
	18:00-19:00	Annette Karmiloff-Smith: Genotype Phenotype Relations: Why a Cognitive Developmental Perspective is Crucial
	20:00-	Dinner in small groups
May 3.		MODELS AND DATA
	9:00-13:00	Morning session: Visual organization in WS (Atkinson, Kovács, Jariabková)
	9:00-9:45	Janette Atkinson: Visuo-Spatial Cognition, Attention And Frontal Function In WS Children
	9:45-10:30	Ilona Kovács: Low-level Perceptual Deficit in Williams Syndrome
	10:30-11:00	Coffee break
	11:00-11:45	Katarina Jariabková: Visuo-constructive abilities in individuals with Williams syndrome and Down syndrome as assessed by Rey-Osterrieth Complex Figure
	11:45-12:45	General discussion on vision
	14:00-16:00	Afternoon session: WS and working memory (Jarrold, Racsmány)
	14:00-14:45	Chris Jarrold: Causes and consequences of poor visuo-spatial short-term memory in Williams syndrome
	14:45-15:30	Mihály Racsmány: Dissociated components of working memory and their effects on long-term acquisition in WS
	15:30-16:00	General discussion on memory
	16:00-	Occasional sight seeing
	19:00-	Opera for those who signed up

May 4.

INTERACTIONS

9:00-11:00

Interactions between parents and scientists (Pogány)

13:00-17:30

Afternoon session:

Methods: Life, vision Memory and neuropsychology

13:00 -14:00

Gábor Pogány: Foster the development of self–help consciousness in families of WS children by interacting with researchers(Video about Hungarian WS children)

14:00-15:00

Vision methods (discussion organized By Ilona Kovács)

15:00-16:00

Chris Jarrold Methods of assessing short-term and working memory

16:00-17:00

Language methods (discussion organized by Ágnes Lukács )

Abstracts

Language profiles in WS

Virginia Volterra

Rome Institute of Psychology,

Early linguistic abilities in Italian children with WS

(with M.Cristina Caselli, Olga Capirci, Stefano Vicari, Francesca Tonucci)

Previous studies of linguistic abilities in Italian-speaking children with WS and DS are briefly reviewed. New data on linguistic performance of 6 Italian children with WS between 3 and 6 years are presented and compared with data on linguistic performance of 6 children with DS matched for chronological, mental age and vocabulary development and of 6 normally developing younger children, matched for mental age and vocabulary. The language measures included also a parent report of early phrase structure, a naming test and a Sentence Repetition Test. Analyses revealed that the three groups of children were at the same vocabulary level, but showed different patterns in sentence production and repetition. Children with WS produced more complete sentences, similar to normally developing children at the same mental age, whereas children with DS produced more telegraphic and incomplete sentences. The difference between children with DS and WS was more marked on the repetition task, suggesting that phonological short-term memory may play a greater role when sentence production is measured through repetition.

Particularly interesting is the comparison between the tree groups in the errors produced in the repetition task These results from younger children confirm and extend previous findings with older children and adolescents with WS. They further suggest that the apparently spared linguistic abilities of children with WS could emerge as an artifact of comparisons made with children with DS, whose sentence production competence is more compromised relative to other verbal and non-verbal abilities.

More recent data collected with more children will also be presented and discussed.

Ágnes Lukács

MTA Linguistics Institute, Budapest

Frequency effects in the WS mental lexicon

One of the most often cited characteristics of Williams syndrome is the tendency of WS people to use infrequent or strange words. This everyday observation has been tested in several experimental situations, the findings of which are still contradictory. We present results from a semantic fluency task, in which subjects had to produce as many category examples as they can in one minute. 11 WS subjects and controls matched in vocabulary level were tested in 8 categories: food, drinks, animals, furniture, clothes, musical instruments, things to read, jobs. Our results are in concert with previous findings by Jarrold et al., (2000): WS subjects and controls did not systematically differ in the number of category items produced in 1 minute (with WS subjects producing more items only in the drinks category). Using category norms as frequency measures, we did not find any difference between the two groups in the average frequency of the items, and no differences were found in the number of non-category items they listed as category members either. WS subjects, though, produced more items with zero frequency in three categories: animals, food and drinks, which are probably the most highly structured and richest categories for children. The lack of systematic differences in frequency between the two groups is supported by our previous results from a picture-naming vocabulary task, in which the WS group, just as controls, performed better with frequent words than with infrequent ones. Although further research is necessary, taken together, our results indicate that frequency is an organizing principle of the WS mental lexicon that operates in the same way as in normals, and peculiarities of WS word usage has to be explained by some other factor.

Michael Thomas

Institute of Child Health, London

Computational modeling of language acquisition in Williams syndrome:
The case of past tense formation.

Connectionist models provide a tool to explore processes of cognitive development, and by extension, atypical development found in developmental disorders. Language development in Williams syndrome (WS) has been characterized as demonstrating selective deficits, with grammatical processes developing normally but processes relating to word-specific knowledge showing deficits. Selective deficits for irregular verbs in English past tense acquisition have been cited as evidence supporting this claim. WS has then been used to make theoretical claims about the structure of the normal language system. In this talk I will describe the most recent empirical evidence on past tense acquisition in WS. I will then introduce a connectionist model of past tense acquisition, and demonstrate how atypical computational constraints in the start state of the model may allow the model to capture the WS profile of past tense acquisition. The model will be used to evaluate the viability of five hypotheses of how language development in WS may be atypical, identified from an extensive review of the literature. The model suggests several constraints may be atypical in the WS language system.

Farrell Ackerman

Department of Linguistics, UC San Diego

Pausing to consider: A lexicalist appraisal of dual mechanism models and Williams Syndrome research in morphology

Over the past decade proponents of the so-called Dual Mechanism model (Pinker 1999, among others) as well as their opponents (most relevantly, Thomas et. al. 2001) have investigated a small number of inflectional phenomena, and generated a large debate. For example, in the domain of German and English plural number marking the former have claimed that the contrast between regular plural inflection versus irregular plural inflection internal to compounding, * rats eater mice eater, respectively, reflects the rule-based default status of the regular plural and the stored lexical status of the irregular plural. Roughly, the claim is that rule generated words are not stored in the lexicon and therefore cannot serve as input to lexical operations such as compounding, while the lexically stored status of irregulars makes them possible candidates for lexical operations such as compounding.

Methodologically the logic of the research among proponents of the dual mechanism approach is clear. Experimental results in several domains of inflectional morphology (past tense, plural marking, regular inflection-inside compounding, comparatives) among unimpaired populations are provided with a theoretical explanation in terms of an opposition between rule-based explanation for regular formations versus an associative-based explanation for irregular formations. The same phenomena are then investigated within special populations where observed dissociations are adduced as evidence for the theoretical division of labor between rules and analogy. This basic strategy has been applied by Clahsen & Almazan 2001 to compounding and Clahsen and Almazan to appear to comparatives in English William Syndrome populations. I will examine issues concerning (ir)regular plural marking, (ir)regular plural marking internal to compounds, and comparative formation since they are phenomena utilized in experiments with William Syndrome populations. Specifically, I will show that experimental and diary data from Finnish first language acquisition demonstrate that, contra expectations of the dual mechanism model, there is regular case inflection internal to compounding, as in Hungarian. Among other things, this suggests that complete and detailed analyses of the relevant phenomena in a wide scatter of languages are crucial before one can draw reliable inferences from their behavior in either unimpaired or special populations. I then look at some derivational operations, particularly with respect to predicate formation operations in Hungarian (see Clahsen et. al. ms. for an intriguing comparison of inflection and derivation among competing morphological models) and argue that theories of morphology in both inflection and derivation need to provide principled ways of capturing gradient degrees of productivity and regularity. Promising lines of inquiry will be drawn from proposals by Jackendoff 1975, Bochner 1993, and Riehemann 1999, 2001. In all instances I try to provide research questions for further study of morphology within the Hungarian Williams Syndrome population (Pléh & Lukács to appear). In this connection, I will suggest that the debate between advocates of a dual mechanism approach and advocates of a single mechanism has been artificially narrow, neglecting, for the most part, instructive research from other traditions in which considerable effort has gone into elaborating the structure of the lexicon and in which morphology is regarded to be a distinct domain, irreducible to either syntactic or phonological principles. I take a perspective on the relevant issues less familiar to psycholinguists and neurolinguists: a unification-based lexicalist perspective on grammar (LEXICAL FUNCTIONAL GRAMMAR (Bresnan 2001, Dalrymple 2001, HEAD DRIVEN PHRASE STRUCTURE GRAMMAR (Sag and Wasow 1999), and CONSTRUCTION GRAMMAR (Fillmore and Kay ms. Ginzburg and Sag 2001) and a inferential-realization perspective on morphology (Robbins 1959, Matthews 1972, Zwicky 1989, Anderson 1992, Aronoff 1994, Spencer 2001, Stump 2001, among others). By and large, in such models the lexicon is the locus of richer structure (networks of related inflected and derived words) and greater explanatory force than is assumed in theories where the lexicon is simply viewed as a repository of listed elements.

Sociality: WS and autism

Miklós Gy?ri

ELTE, Institute of Psychology, Autism Foundation, Budapest,

Fractionating 'theory of mind': evidence from autism. An overview.

Clinical picture of Williams syndrome (WS) involves high level of sociability, in sharp contrast to autism (autistic spectrum disorders; ASD). Recent studies, however, suggest that 'theory of mind', the highly complex human cognitive capacity to attribute mental states to agents, shows a specific pattern of relative impairment in individuals with WS. These findings render research on theory of mind functioning in ASD especially relevant in the context of WS, since marked impairment of theory of mind is well documented in ASD, as well as its causal role in the variety of social difficulties observable in these syndromes.

Empirical studies on theory of mind functioning in ASD revealed a considerable variety in the pattern, degree and developmental changes in theory of mind impairment. These results – in line with others from neurotypical subjects – suggest that typically functioning theory of mind capacity is underlain by multiple neurocognitive systems that may dissociate from each other in atypical development.

The present contribution overviews and evaluates the most important evidence from ASD for such dissociations, focusing on the following issues:

Theory of mind capacity and the variety of social behaviours
Competence and performance in theory of mind functioning
Components of theory of mind competence
Developmental patterns and ways of compensation

Zsuzsa Káldy

Rutgers, Department of Psychology,

What mechanisms affect the unusual social profile of Williams Syndrome individuals?

with Alan Leslie

Individuals with Williams Syndrome (WS) show an unusual pattern of social behavior. They are overfriendly and outgoing, and their language use is notably affective (Bellugi et al, 1998). These characteristics are the opposite of what we find in autism, where one of the core deficits is in mentalizing (Theory of Mind, ToM). However, WS individuals are not super-mentalizers: they also have some serious social difficulties. In terms of age groups, infants and toddlers do not follow the gaze of others (joint attention), children cannot establish contact with their peers, and in experimental situations (ToM tasks) WS adults do not always reach the level of normal children. We hypothesize that what is spared in WS is a paradoxical “conversational module”. A similar phenomenon was found in a case of spina bifida many years ago by Cromer. The mechanism of ToM might be independent from this module.

Determinants of development in WS (Public Session)

Ursula Bellugi, Edward S. Klima

Laboratory for Cognitive Neuroscience, The Salk Institute for Biological Studies,

La Jolla, CA

Linking Cognitive Neuroscience and Molecular Genetics:
New Perspectives from Williams Syndrome

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.

Annette Karmiloff-Smith

Institute of Child Health, London,

Genotype Phenotype Relations:
Why a Cognitive Developmental Perspective is Crucial

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.

WS and working memory

Chris Jarrold

Bristol University, Department of Psychology,

Causes and consequences of poor visuo-spatial short-term memory in Williams syndrome

Individuals with Williams syndrome perform poorly on tests of visuo-spatial short-term memory, such as the Corsi span task, in which participants have to reproduce spatial sequences in correct serial order. In our work my colleagues and I have explored possible causes of this apparent problem, and in particular, have asked whether individuals with Williams syndrome suffer from a fundamental impairment to a specific visuo-spatial short-term store. In fact our evidence to support this view is mixed, and it remains unclear whether people with Williams syndrome have poorer visuo-spatial short-term memory than one would expect given their general problems in the visuo-spatial domain. In an alternative approach to this issue we have examined performance in domains that one might expect to be affected by a visuo-spatial short-term memory deficit. Although we find no evidence of particular problems in learning visuo-spatial associations, we have found evidence of impaired comprehension of spatial descriptions in Williams syndrome. This deficit might result from an underlying visuo-spatial short-term memory problem, but alternative explanations of this finding will also be discussed.

Mihály Racsmány

SZTE Szeged

Dissociated components of working memory and their effects on long-term acquisition in WS

With Á. Lukács, Cs. Pléh, I. Kovács

In the past decade Williams syndrome (WS) has been linked to a cognitive profile characterized by relatively preserved language abilities and seriously injured spatial cognition (see Bellugi et al., 2000). One candidate as a background mechanism for diverging verbal and spatial abilities is a multi-component working memory system (Baddeley, 1986). Jarrold et al (1999) found dissociated working memory performance in WS with a relatively preserved verbal and an impaired spatial short-term memory capacity. Grant et al (1997) found strong relationship between WS children's verbal working memory and language performances.

In four experiments we tried to test the effect of working memory capacity on long-term verbal and spatial acquisition. In Experiment 1 we replicated the result of Jarrold et al (1999) as we found dissociated verbal (digit span) and spatial (Corsi blocks) working memory capacities in WS children comparing their performance to control groups matched either on verbal or spatial mental age. In Experiment 2 we investigated the relationship between verbal working memory capacity and productive vocabulary knowledge. According to our results, verbal working memory span is a much stronger predictor of vocabulary knowledge in WS than in the control group matched on verbal mental age. In Experiment 3 we tested WS children's spatial learning abilities using a repeated localization task (Rey 5/25) and a map-route learning task. We found evidence that WS children show impaired spatial learning on both tasks compared to control groups matched on spatial mental age (block design) and their learning performance was predictable by spatial working memory capacity. Finally, in Experiment 4, similarly to Jarrold et al (in press), we found spatial language difficulties using productive tasks and this performance was influenced both by verbal and spatial working memory capacities. Our results support a view that individual differences in working memory capacity constrain the rate and speed of long-term acquisition of verbal and spatial information.

Visual organization in WS

Janette Atkinson

University College London, Department of Psychology,

Visuo-Spatial Cognition, Attention And Frontal Function In WS Children

With Oliver Braddick, Shirley Anker, Marko Nardini

Visual information is processed in two main brain circuits (streams) called the ‘ventral’ (for discriminating between objects and recognizing faces - who? what?) and ‘dorsal’ (for analyzing relative motion and position in space to generate actions - where? how?). Our studies with young individuals with WS has led us to the general hypothesis of ‘dorsal stream vulnerability’ where certain brain circuits, within the parietal and frontal lobes together with the cerebellum, develop atypically. Support for this model comes from our studies with a large number of WS individuals (N =120) across a wide age range (9 months to 16 years), from multiple measures of vision (including stereopsis for depth perception) and spatial cognition e.g. tests to separate dorsal and ventral stream function (block construction matching and copying, adaptation of Goodale post-box task, motion and form coherence thresholds) and frontal lobe function (counter pointing, Piagetian object permanence ‘A not B with delay’, Russell Box task, Diamond’s Day/Night verbal ‘Stroop’, motor planning tasks). We find performance divided between WS individuals who have difficulties on both verbal and visuospatial tasks and those with difficulties only on spatial tasks. Remarkable variability within and across individuals across tests is a common feature, including general problems of attention in most, but not all, WS children. These attentional difficulties are of particular importance for educational/ intervention schemes. We discuss possible brain underpinnings of these intra and inter individual differences in relation to spatial and attentional tasks.

Methods: We report initial findings on (a) a virtual reality navigational task, in which children locate objects following spatial reorientation (adapted from a task of O’ Keefe and Burgess to test adult hippocampal function). We link results to our earlier findings on spatial planning. (b) 4 subtests of the TEACh battery (‘Test of Everyday Attention in Children’) comparing function of different attentional systems (for auditory sustained attention, visual search and inhibition of a prepotent response).

Results: In the virtual reality task, two inter-related difficulties have been identified; the ability to switch between spatial representations at different locations and to translate 2D into 3D spatial representations. On TEACh, WS individuals (aged 5-14 years) show a varying degree of delay across all tests, with the auditory, sustained attention, monitoring task more difficult than tests of selective attention (involving pattern matching). Relative differences in difficulty may depend on particular test material, the modality used and /or memorial or temporal factors.

Discussion: In the light of these new results we reconsider and refine the ‘dorsal stream vulnerability’ model and address the potential use of such tasks as educational and strategic training games to aid WS individuals in everyday life.

Ilona Kovács

Rutgers Department of Psychology,

Low-level Perceptual Deficit in Williams Syndrome

With Á. Lukács, Á Fehér, M. Racsmány, Cs. Pléh

The most severe volumetric changes in the WMS brain are found in the occipital lobe, as measured by gross anatomical techniques (Reiss et al, 2000). Occipital involvement is indicated also by cytoarchitectonic measurements: while most cortical areas appear relatively normal, signs of abnormal neural connectivity have been discovered in area 17 (Galaburda & Bellugi, 2000). These anatomical findings seem to call for studies addressing early levels of visual processing in WMS.

We studied early visual cortical processing in WMS by employing an orientation discrimination, and a contour integration task. The orientation discrimination task involves judgments about the spatial arrangement of simultaneously presented pairs of Gabor signals, and it is assumed to be mediated by relatively local, short-range spatial interactions. The contour detection task involves the detection of a closed chain of high contrast Gabor signals embedded in a random background of Gabor signals, and it is believed to be mediated by long-range spatial interactions. Typically developing children show an extended developmental period in their ability to integrate orientation information across the visual field in both tasks (Nolan and Kovacs, 2002; Kovacs et al, 1999). WMS subjects in our study lag behind typical age-norms, and never reach typical adult performance levels. Some WMS subjects also deviate from the normal pattern of results in orientation discrimination, presenting the lack of the “oblique effect.” These results support the possibility of abnormal spatial interactions and neural connectivity in WMS visual cortex. The correlation between anatomical and behavioral data in this case opens up a new dimension in the understanding of the neuroperceptual profile in WMS; and it predicts that WMS subjects will encounter difficulties in tasks involving both short-range and long-range interactions, and contextual integration.

Katarina Jariabková

Department of Social and Biological Communication, Slovak Academy of Sciences,

Visuo-constructive abilities in individuals with Williams syndrome and Down syndrome as assessed by Rey-Osterrieth Complex Figure

With V. Bzdúch ², M. Koš? ³, M. Šustrová ⁴

²First Department of Pediatrics, University Children’s Hospital, ³Department of Psychology, Trnava University, ⁴Institute of Preventive and Clinical Medicine, Bratislava, Slovakia

Visuo-constructive impairments in copying and free drawings have been reported in both Williams syndrome (WS) and Down syndrome (DS). Qualitatively, productions of subjects with WS often show poor cohesion and lack overall organization (cf. Wang et al., 1995; Bellugi et al., 2000).

The copy task of the Rey-Osterrieth Complex Figure (ROCF) permits an analysis of performance accuracy as well as of the drawing procedure (Osterrieth, 1944). The purpose of the paper is to present productions of WS and DS subjects in the ROCF and to discuss the use of the test as a tool for assessing the visuo-constructive abilities in WS.

The task was administered to 20 WS subjects (mean age 16;10, range 7 to 33 years) and 12 DS subjects (mean age 17;7, range 7 to 28 years). The accuracy scores of all WS subjects and of most of the DS subjects (83%) fell below the 10th percentile of developmental norms. However, 2 DS subjects performed within the average range of the norms. Types of drawing procedures with little or no organization were observed in subjects from both groups. Three of the DS subjects (25%) demonstrated developmentally higher procedures typical of normal adults. No WS subject reached this level.

The data indicate more severe impairments in the WS group and point to possible differences in performance with regard to the way of administration of the test.

Interactions between parents and scientists

Gábor Pogány

Semmelweis Med University, Budapest and Hungarian Williams Syndrome, Association,

Foster the development of self–help consciousness in families of WS children by interacting with researchers

We want to present the activity of the Hungarian Williams Syndrome Association, introducing our specialties and the main differences between our organization and the other similar associations in the western countries.

Because of the lack of appropriate state education and health care system, we need to undertake several functions of the government. Therefore, we need to organize „gap-filling” services like hydrotherapeutic and rehabilitation gymnastic (HRG) and sensomotor training, special educational therapy, music and art therapy, special language and music teaching. The basis of our programs is the good connection and fruitful cooperation with professionals and researchers. A demonstration of our regular programs will be held ensuring the possibilities of communication between parents and the participating researchers.

We have some good collaboration with WS investigators. For example, ours is the first psychological study aiming at the characteristics of cognitive mechanisms of WS children in Hungary. The neuropsychological methods developed by the researchers can also introduce new diagnostic tools for handicapped children.

Results will hopefully open new possibilities of training and rehabilitation: locating the specific deficit within the cognitive domain is the first step in choosing or developing the appropriate rehabilitative method. We would also like to draw attention to the educational and psychological problems WS children and their families have to face.

The Hungarian Williams Syndrome Association is happy to further help the parents and teachers with new information based on psychological testing of the children. Most importantly, by informing teachers about the specific peaks and valleys in the cognitive and behavioral profile of WS people. We might facilitate the development of more differentiated teaching methods in a classroom of mentally retarded children using new information of their learning abilities in different domains.

Methods Measuring vision Memory measures and neuropsychology

Chris Jarrold

Bristol University, Department of Psychology

Methods of assessing short-term and working memory

In this discussion of methodological approaches I will outline ways of assessing short-term and working memory. In addition, I will attempt to show how methodological considerations arise from three particular theoretical assumptions.

These are,

i)that working memory differs from short-term memory in terms of processing requirements,

ii)that verbal short-term memory performance is closely linked to language ability, and

iii)that visuo-spatial short-term memory might fractionate into separate visual and spatial subcomponents.

List of Participants

Speakers

Focus Group Of Collegium Budapest

Research Associates

Overview of the program