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1

PERSONALITY CHARACTERISTICS OF ADOLESCENTS AND ADULTS WITH WILLIAMS SYNDROME AND DOWN SYNDROME

100%
Jariabkova K., Ruisel I., Andreanska V., Bzduch V.
Studia Psychologica
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2006
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vol. 48
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issue 4
361-370
EN
This study presents an attempt to approach the personality characteristics of the adolescents and adults with Williams syndrome and those with Down syndrome from the perspective of the Big Five personality dimensions. The parents of 22 individuals with Williams syndrome (age range 14 to 37 years) and 22 individuals with Down syndrome (age range 14 to 38 years) as well as the parents of those from two chronological age- and gender-matched control groups from a nonclinical population completed the FFI-MH questionnaire (author I. Ruisel). The results showed lower openness to the experience in both the Williams syndrome and the Down syndrome groups than in their control groups. The Williams syndrome group was assessed higher on extroversion and neuroticism and lower on conscientiousness than its control group as well as the Down syndrome group. The agreeableness did not differ across groups. The data are discussed within the framework of the results from the previous research.
2

NUMERICAL ABILITIES IN WILLIAMS-SYNDROME: DISSOCIATED ANALOGUE QUANTITATIVE SYSTEM AND VERBAL RECALL

100%
Krajcsi A., Igacs J., Lukacs A., Racsmany M., Pleh C.
Magyar Pszichologiai Szemle (Hungarian Psychological Review)
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2008
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vol. 63
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issue 1
103-115
EN
Williams syndrome (WS) is a rare genetic disorder accompanied by serious damage to numerical abilities. The working of two numerical systems, the analogue amount system and the verbal system, were investigated in Williams syndrome. Three different numerical tasks were used: simple addition, simple multiplication, and number comparison. A new matching method was introduced to find a proper control group. Typically developing control groups were matched using reaction time data from one of the tasks. The WS group had a relatively fast reaction time in addition and multiplication tasks (verbal recall of the number table), but the group was slow in number comparison (analogue quantity estimation in the intraparietal sulcus). In the number comparison task no reverse number effects were found, and the distance effect was stronger then in the control groups. On the basis of these data the postulation of an impaired analogue quantity system together with a relatively intact verbal recall system in Williams syndrome is supported.
3

SPACE AND THE WORLD OF LANGUAGE

88%
Pleh C.
Magyar Pszichologiai Szemle (Hungarian Psychological Review)
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2007
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vol. 62
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issue 3
279-312
EN
Modern psycholinguistic studies started to use experimental and child language observational data on spatial language to obtain evidence for the primacy issue: what leads, language or spatial cognition in the articulation of spatial language. The author's studies on Hungarian, a language characterized by rich agglutination and an articulate spatial language system follow this pattern. The author was particularly interested to see how the unfolding and impairment of the spatial language system supports claims of universality in a language where some conceptual distinctions are particularly easy to observe due to the clear and obligatory marking. Hungarian data illustrate how a universal cognitive tendency - the primacy of goals - can be shown to exist very early on in a language that requires distinctions along the path (e.g. in, into,'from inside'). This tendency was shown in learning artificial space markers as well, and it was present both in normal and in developmentally impaired populations, notably in Williams syndrome. At the same time, probably due to their impairments to the parietal lobe, Williams syndrome subjects do show a lower performance in spatial language. However, this lower performance follows the same pattern as in typically developing subjects. The paper tries to relate the different approaches, namely psychological, linguistic, developmental, neuroscience, and evolutionary approaches to understand the complexity of coding spatial distinctions in language.
4

SPATIAL MEMORY FOR REFERENCE POINTS AND PATHS IN WILLIAMS SYNDROME: THE MAP TASK

88%
Racsmany M., Lukacs A., Pleh C.
Magyar Pszichologiai Szemle (Hungarian Psychological Review)
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2008
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vol. 63
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issue 1
87-102
EN
Williams-syndrome is a rare genetic disorder characterized by a specific developmental profile following damage to a sequence on chromosome 7. A basic feature of the WS cognitive profile is assumed to be a dissociation between verbal and spatial abilities. In the authors' studies spatial memory performance of WS children was compared with three matched control groups. These studies allow for the analysis of several methodological and theoretical issues concerning spatial memory. The central issue in their studies was the attempt to elaborate a new task to measure spatial memory based on ecologically familiar tasks. This is the so called Map task requiring long term learning of spatial reference points and paths. Using the Map task they were able to show impaired performance in subjects with Williams syndrome both in acquiring reference points and paths. Spatial working memory capacity had a clear influence on long term spatial learning. However, retention of reference points had a stronger relationship to working memory capacity than performance regarding paths. On the whole, the Map task seems to be a useful procedure to investigate the development of spatial memory abilities.
5

CHANGES IN THE USE OF SPATIAL FRAMES OF REFERENCE IN TYPICALLY DEVELOPING AND WILLIAMS SYNDROME CHILDREN

75%
Ivady R. E., Felhosi G., Pleh C.
Magyar Pszichologiai Szemle (Hungarian Psychological Review)
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2008
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vol. 63
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issue 1
213-250
EN
This study aimed to reveal the relationship between language and thinking, more specifically the linguistic relativism theory as formulated by Levinson (1996) and colleagues (Pederson, Danziger et al., 1998). The authors examined developmental change in the use of frames of reference in five typically developing groups (3-10 years) and a Williams syndrome group (7-21 years). Williams syndrome children were chosen to tease apart the different effects of language (relatively unimpaired) and cognition (mildly/severly impaired). Participants had to memorize a spatial array, then turn 180° and choose between two arrays, differing only in their frame of reference (relative or absolute). The authors also administered various tests (TROG, RAVEN, Corsi, RMET) to examine individual differences. The developmental trend was exactly the opposite of the hypothesis posed by Levinson: speakers of an inherently intrinsic/relative language seemed to prefer absolute choices increasingly with age. Yet a connection between language and reference frames could not be established by way of a correlation between administered language proficiency tests and preferred frame of reference. Williams syndrome children showed radically different preferences in the test, so we must conclude that their relatively impaired language could not give them a crutch in the task. The authors conclude that probably there is a threefold causality in the choice of reference frame in children: 1. Biological effects (such as sex and handedness) and 2. individual differences (such as intelligence and spatial memory) have a large impact on spatial reference choices (possibly only in childhood). 3. Both immediate (visible allocentric and egocentric cues) and general environment (education, culture) are important, yet language alone does seem to play a role.
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