Genetic contributions to visuospatial cognition in Williams syndrome: insights from two contrasting partial deletion patients

Background Williams syndrome (WS) is a rare neurodevelopmental disorder arising from a hemizygotic deletion of approximately 27 genes on chromosome 7, at locus 7q11.23. WS is characterised by an uneven cognitive profile, with serious deficits in visuospatial tasks in comparison to relatively proficient performance in some other cognitive domains such as language and face processing. Individuals with partial genetic deletions within the WS critical region (WSCR) have provided insights into the contribution of specific genes to this complex phenotype. However, the combinatorial effects of different genes remain elusive. Methods We report on visuospatial cognition in two individuals with contrasting partial deletions in the WSCR: one female (HR), aged 11 years 9 months, with haploinsufficiency for 24 of the WS genes (up to GTF2IRD1), and one male (JB), aged 14 years 2 months, with the three most telomeric genes within the WSCR deleted, or partially deleted. Results Our in-depth phenotyping of the visuospatial domain from table-top psychometric, and small- and large-scale experimental tasks reveal a profile in HR in line with typically developing controls, albeit with some atypical features. These data are contrasted with patient JB’s atypical profile of strengths and weaknesses across the visuospatial domain, as well as with more substantial visuospatial deficits in individuals with the full WS deletion. Conclusions Our findings point to the contribution of specific genes to spatial processing difficulties associated with WS, highlighting the multifaceted nature of spatial cognition and the divergent effects of genetic deletions within the WSCR on different components of visuospatial ability. The importance of general transcription factors at the telomeric end of the WSCR, and their combinatorial effects on the WS visuospatial phenotype are also discussed

Implicit Measures of Lostness and Success in Web Navigation

In two studies, we investigated the ability of a variety of structural and temporal measures computed from a web navigation path to predict lostness and task success. The user’s task was to find requested target information on specified websites. The web navigation measures were based on counts of visits to web pages and other statistical properties of the web usage graph (such as compactness, stratum, and similarity to the optimal path). Subjective lostness was best predicted by similarity to the optimal path and time on task. The best overall predictor of success on individual tasks was similarity to the optimal path, but other predictors were sometimes superior depending on the particular web navigation task. These measures can be used to diagnose user navigational problems and to help identify problems in website design

How users assess web pages for information-seeking

In this paper, we investigate the criteria used by online searchers when assessing the relevance of web pages for information-seeking tasks. Twenty four participants were given three tasks each, and indicated the features of web pages which they employed when deciding about the usefulness of the pages in relation to the tasks. These tasks were presented within the context of a simulated work-task situation. We investigated the relative utility of features identified by participants (web page content,structure and quality), and how the importance of these features is affected by the type of information-seeking task performed and the stage of the search. The results of this study provide a set of criteria used by searchers to decide about the utility of web pages for different types of tasks. Such criteria can have implications for the design of systems that use or recommend web pages

The relationship of (perceived) epistemic cognition to interaction with resources on the internet

Information seeking and processing are key literacy practices. However, they are activities that students, across a range of ages, struggle with. These information seeking processes can be viewed through the lens of epistemic cognition: beliefs regarding the source, justification, complexity, and certainty of knowledge. In the research reported in this article we build on established research in this area, which has typically used self-report psychometric and behavior data, and information seeking tasks involving closed-document sets. We take a novel approach in applying established self-report measures to a large-scale, naturalistic, study environment, pointing to the potential of analysis of dialogue, web-navigation – including sites visited – and other trace data, to support more traditional self-report mechanisms. Our analysis suggests that prior work demonstrating relationships between self-report indicators is not paralleled in investigation of the hypothesized relationships between self-report and trace-indicators. However, there are clear epistemic features of this trace data. The article thus demonstrates the potential of behavioral learning analytic data in understanding how epistemic cognition is brought to bear in rich information seeking and processing tasks

Manager's degree of JIT involvement, locus of control and managerial performance

The competitive global environment has lead many firms into adopting practices that focus on eliminating inefficiencies across the enterprise and its supply chain. The Just-in-Time philosophy is one such practice, however, research has predominantly focused on its technical features and on organisational variables, with surprising little research at the individual level. This paper examines JIT at an individual level and argues that the manager’s locus of control orientation would interact with their degree of JIT involvement to affect managerial performance. The results of a survey of 60 managers employing JIT, demonstrate that an increased degree of JIT involvement leads to a more positive effect on managerial performance for internal locus of control managers than for external locus of control managers

Evaluation of a prototype interface for structured document retrieval

Document collections often display either internal structure, in the form of the logical arrangement of document components, or external structure, in the form of links between documents. Structured document retrieval systems aim to exploit this structural information to provide users with more effective access to structured documents. To do this, the associated interface must both represent this information explicitly and support users in their browsing behaviour. This paper describes the implementation and user-centred evaluation of a prototype interface, the RelevanceLinkBar interface. The results of the evaluation show that the RelevanceLinkBar interface supported users in their browsing behaviour, allowing them to find more relevant documents, and was strongly preferred over a standard results interface

Employee Age as a Moderator of the Relationship Between Ambition and Work Role Affect

Past research has demonstrated a negative relationship between ambition, or the desire to get ahead, and job satisfaction. In the present paper, age was hypothesized to moderate the relationship between ambition and job satisfaction such that the relationship between ambition and satisfaction is more negative for older employees than for younger employees. Three studies, with three criterion variables (promotion satisfaction, extrinsic job satisfaction, overall job satisfaction), were used to test the hypothesis. Results indicated support for the hypothesized interaction. The discussion focuses on the implications of the results for organizational and individual career management strategies

Proceedings ICPW'07: 2nd International Conference on the Pragmatic Web, 22-23 Oct. 2007, Tilburg: NL

Proceedings ICPW'07: 2nd International Conference on the Pragmatic Web, 22-23 Oct. 2007, Tilburg: N