Neural processes underlying conceptualization in language production

Conceptualization, speech production, ERPs, fMRI

Screening for Fabry Disease in Left Ventricular Hypertrophy: Documentation of a Novel Mutation

AbstractBackground:Fabry disease is a lysosomal storage disease caused by enzyme α-galactosidase A deficiency as a result of mutations in the GLA gene. Cardiac involvement is characterized by progressive left ventricular hypertrophy.Objective:To estimate the prevalence of Fabry disease in a population with left ventricular hypertrophy.Methods:The patients were assessed for the presence of left ventricular hypertrophy defined as a left ventricular mass index ≥ 96 g/m2 for women or ≥ 116 g/m2 for men. Severe aortic stenosis and arterial hypertension with mild left ventricular hypertrophy were exclusion criteria. All patients included were assessed for enzyme α-galactosidase A activity using dry spot testing. Genetic study was performed whenever the enzyme activity was decreased.Results:A total of 47 patients with a mean left ventricular mass index of 141.1 g/m2 (± 28.5; 99.2 to 228.5 g/m2] were included. Most of the patients were females (51.1%). Nine (19.1%) showed decreased α-galactosidase A activity, but only one positive genetic test − [GLA] c.785G>T; p.W262L (exon 5), a mutation not previously described in the literature. This clinical investigation was able to establish the association between the mutation and the clinical presentation.Conclusion:In a population of patients with left ventricular hypertrophy, we documented a Fabry disease prevalence of 2.1%. This novel case was defined in the sequence of a mutation of unknown meaning in the GLA gene with further pathogenicity study. Thus, this study permitted the definition of a novel causal mutation for Fabry disease - [GLA] c.785G>T; p.W262L (exon 5).

Static and dynamic interpretations of hand specific body language cues for HCI

Otto-von-Guericke-Universität Magdeburg, Fakultät für Elektrotechnik und Informationstechnik, Univ., Dissertation, 2015

Defining the criteria for the evaluation of self-study multimedia language learning materials: do they facilitate or inhibit learner autonomy?

Durant els darrers anys, s’han publicat un gran nombre de materials multimèdia destinats a l’aprenentatge de llengües, la major part dels quals son CD-ROM dissenyats com a cursos per l’autoaprenentatge. Amb aquests materials, els alumnes poden treballar independentment sense l’assessorament d’un professor, i per aquest motiu s’ha afirmat que promouen i faciliten l’aprenentatge autònom. Aquesta relació, però, no es certa, com Phil Benson i Peter Voller 1997:10) han manifestat encertadament:(…) Such claims are often dubious, however, because of the limited range of options and roles offered to the learner. Nevertheless, technologies of education in the broadest sense can be considered to be either more or less supportive of autonomy. The question is what kind of criteria do we apply in evaluating them? En aquest article presentem una investigació conjunta on es defineixen els criteris que poden ser utilitzats per avaluar materials multimèdia en relació a la seva facilitat per permetre l’aprenentatge autònom. Aquests criteris son la base d’un qüestionari que s’ha emprat per avaluar una selecció de CD-ROM destinats a l’autoaprenentatge de llengües. La estructura d’aquest article és la següent: - Una introducció de l’estudi - Els criteris que s’han utilitzar per la creació del qüestionari - Els resultats generals de l’avaluació - Les conclusions que s’han extret i la seva importància pel disseny instructiu multimèdia

Investigating independent language learning in classroom-based EFL Learners

La recerca efectuada sobre les estratègies d’aprenentatge de llengües ha demostrat que els aprenents que utilitzen estratègies metacognitives (planificació, revisió i avaluació) desenvolupen estratègies cognitives més eficaces (Anderson, 2002). Aquest article descriu les activitats que 43 estudiants de llengua estrangera de la Universitat de Vic van emprendre de forma independent i dedueix les estratègies metacognitives que van utilitzar sense cap formació prèvia en estratègies. Els estudiants van completar un dossier on expressaven les necessitats d’aprenentatge, la planificació i supervisió de les activitats i finalment l’avaluació de l’aprenentatge que havien portat a terme de manera independent fora de les hores lectives. La primera fase de l’anàlisi de les dades revela que, tot i que els estudiants foren capaços d’expressar les necessitats d’aprenentatge en general, la formulació d’objectius i la supervisió de les activitats fou escassa. La discussió gira entorn de la formació dels estudiants de llengües estrangeres en estratègies metacognitives i la integració de l’aprenentatge autònom dins el currículum docent.

Eliciting and fostering learners' metacognitive knowledge about language learning in self-directed learning programs: a review of data collection methods and procedures

Són molts els estudis que avui en dia incideixen en la necessitat d’oferir un suport metodològic i psicològic als aprenents que treballen de manera autònoma. L’objectiu d’aquest suport és ajudar-los a desenvolupar les destreses que necessiten per dirigir el seu aprenentatge així com una actitud positiva i una major conscienciació envers aquest aprenentatge. En definitiva, aquests dos tipus de preparació es consideren essencials per ajudar els aprenents a esdevenir més autònoms i més eficients en el seu propi aprenentatge. Malgrat això, si bé és freqüent trobar estudis que exemplifiquen aplicacions del suport metodològic dins els seus programes, principalment en la formació d’estratègies o ajudant els aprenents a desenvolupar un pla de treball, aquest no és el cas quan es tracta de la seva preparació psicològica. Amb rares excepcions, trobem estudis que documentin com s’incideix en les actituds i en les creences dels aprenents, també coneguts com a coneixement metacognitiu (CM), en programes que fomenten l’autonomia en l’aprenentatge. Els objectius d’aquest treball son dos: a) oferir una revisió d’estudis que han utilitzat diferents mitjans per incidir en el CM dels aprenents i b) descriure les febleses i avantatges dels procediments i instruments que utilitzen, tal com han estat valorats en estudis de recerca, ja que ens permetrà establir criteris objectius sobre com i quan utilitzar-los en programes que fomentin l’aprenentatge autodirigit.

The Third Language in Dubbing

Si tenim en compte que el procés de traducció és un procés complex, el procés de traducció d'un text audiovisual encara ho és més. A part dels problemes tècnics com per exemple la sincronització labial i temporal hi ha altres factors a tenir en compte com és l'ús d'estructures lingüístiques i textuals específiques d'aquest canal de comunicació. Moltes de les pel·lícules que sovint podem veure a la pantalla estan produïdes als Estats Units, i per tant estan en anglès, i hi ha una necessitat imperiosa de traduir-les al català i castellà. A part d'això, en algunes ocasions el text audiovisual original conté més d'una llengua; aquest és el cas de Raid on Rommel i Butch Cassidy and the Sundance Kid, entre d'altres. En aquests casos, ens trobem amb una nova dificultat: el traductor ha de traduir aquesta tercera llengua (o dialecte) a la corresponent cultura d'arribada. Aquest article vol presentar i exposar alguns exemples d'aquest tipus de dificultats així com també les diferents solucions que s'han aportat davant una "tercera" llengua.

Documentation and sport information: the challenges and opportunities brought by new technologies

This text corresponds to the contents of the seminar “Information Services at the Sport Institutions” held by the author as part of the programme of activities of the Invited Professor of Olympism of the International Chair in Olympism (IOC-UAB) in 2001. The seminar discusses the potential of technologies such as the Internet in sports documentation.

Validity, reliability and responsiveness of the French language translation of the Western Ontario Shoulder Instability Index (WOSI).

BACKGROUND: The WOSI (Western Ontario Shoulder Instability Index) is a self-administered quality of life questionnaire designed to be used as a primary outcome measure in clinical trials on shoulder instability, as well as to measure the effect of an intervention on any particular patient. It is validated and is reliable and sensitive. As it is designed to measure subjective outcome, it is important that translation should be methodologically rigorous, as it is subject to both linguistic and cultural interpretation. OBJECTIVE: To produce a French language version of the WOSI that is culturally adapted to both European and North American French-speaking populations. MATERIALS AND METHODS: A validated protocol was used to create a French language WOSI questionnaire (WOSI-Fr) that would be culturally acceptable for both European and North American French-speaking populations. Reliability and responsiveness analyses were carried out, and the WOSI-Fr was compared to the F-QuickDASH-D/S (Disability of the Arm, Shoulder and Hand-French translation), and Walch-Duplay scores. RESULTS: A French language version of the WOSI (WOSI-Fr) was accepted by a multinational committee. The WOSI-Fr was then validated using a total of 144 native French-speaking subjects from Canada and Switzerland. Comparison of results on two WOSI-Fr questionnaires completed at a mean interval of 16 days showed that the WOSI-Fr had strong reliability, with a Pearson and interclass correlation of r=0.85 (P=0.01) and ICC=0.84 [95% CI=0.78-0.88]. Responsiveness, at a mean 378.9 days after surgical intervention, showed strong correlation with that of the F-QuickDASH-D/S, with r=0.67 (P<0.01). Moreover, a standardized response means analysis to calculate effect size for both the WOSI-Fr and the F-QuickDASH-D/S showed that the WOSI-Fr had a significantly greater ability to detect change (SRM 1.55 versus 0.87 for the WOSI-Fr and F-QuickDASH-D/S respectively, P<0.01). The WOSI-Fr showed fair correlation with the Walch-Duplay. DISCUSSION: A French-language translation of the WOSI questionnaire was created and validated for use in both Canadian and Swiss French-speaking populations. This questionnaire will facilitate outcome assessment in French-speaking settings, collaboration in multinational studies and comparison between studies performed in different countries. TYPE OF STUDY: Multicenter cohort study. LEVEL OF EVIDENCE: II.

Gestor d'expedients per l'Institut Municipal d'Hisenda

El projecte recull el treball portat a càrrec per l’anàlisi, disseny i implementació d’una eina per l’Institut Municipal D’Hisenda de l’Ajuntament de Barcelona que compleixi les necessitats d’un sistema d’informació capaç de gestionar els expedients que genera una sèrie de tributs, les sancions que comporten així com la documentació necessària per la comunicació amb el ciutadà. Per realitzar l’aplicació s’han utilitzat tecnologies que ens permeten treballar en l’entorn web, un nucli programat en llenguatge Java sobre la plataforma MVC de Struts, tot sobre un servidor d’aplicacions WebSphere i un motor de base de dades Oracle.

Native language, spoken language, translation and trade

We construct new series for common native language and common spoken language for 195 countries, which we use together with series for common official language and linguis-tic proximity in order to draw inferences about (1) the aggregate impact of all linguistic factors on bilateral trade, (2) whether the linguistic influences come from ethnicity and trust or ease of communication, and (3) in so far they come from ease of communication, to what extent trans-lation and interpreters play a role. The results show that the impact of linguistic factors, all together, is at least twice as great as the usual dummy variable for common language, resting on official language, would say. In addition, ease of communication is far more important than ethnicity and trust. Further, so far as ease of communication is at work, translation and inter-preters are extremely important. Finally, ethnicity and trust come into play largely because of immigrants and their influence is otherwise difficult to detect.

Native language, spoken language, translation and trade

We construct new series for common native language and common spoken language for 195 countries, which we use together with series for common official language and linguis-tic proximity in order to draw inferences about (1) the aggregate impact of all linguistic factors on bilateral trade, (2) whether the linguistic influences come from ethnicity and trust or ease of communication, and (3) in so far they come from ease of communication, to what extent trans-lation and interpreters play a role. The results show that the impact of linguistic factors, all together, is at least twice as great as the usual dummy variable for common language, resting on official language, would say. In addition, ease of communication is far more important than ethnicity and trust. Further, so far as ease of communication is at work, translation and inter-preters are extremely important. Finally, ethnicity and trust come into play largely because of immigrants and their influence is otherwise difficult to detect.

A Framework for Analyzing Language and Welfare

The paper proposes a general model that will encompass trade and social benefits of a common language, a preference for a variety of languages, the fundamental role of translators, an emotional attachment to maternal language, and the threat that globalization poses to the vast majority of languages. With respect to people’s emotional attachment, the model considers minorities to suffer losses from the subordinate status of their language. In addition, the model treats the threat to minority language as coming from the failure of the parents in the minority to transmit their maternal language (durably) to their children. Some familiar results occur. In particular, we encounter the usual social inefficiencies of decentralized solutions to language learning when the sole benefits of the learning are communicative benefits (though translation intervenes). However, these social inefficiencies assume a totally different air when the con-sumer gains of variety are brought in. One fundamental aim of the paper is to bring together contributions to the economics of language from labor economics, network externalities and international trade that are typically treated separately.

Reversible behavioural autistic-like regression: A manifestation of a special (new?) epileptic syndrome in a 28-month-old child. A 2-year longitudinal study .

A 28-month-old boy was referred for acute onset of abnormal head movements. History revealed an insidious progressive regression in behaviour and communication over several months. Head and shoulder 'spasms' with alteration of consciousness and on one occasion ictal laughter were seen. The electroencephalograph (EEG) showed repeated bursts of brief generalized polyspikes and spike-wave during the 'spasms', followed by flattening, a special pattern which never recurred after treatment. Review of family videos showed a single 'minor' identical seizure 6 months previously. Magnetic resonance imaging was normal. Clonazepam brought immediate cessation of seizures, normalization of the EEG and a parallel spectacular improvement in communication, mood and language. Follow-up over the next 10 months showed a new regression unaccompained by recognized seizures, although numerous seizures were discovered during the videotaped neuropsychological examination, when stereotyped subtle brief paroxysmal changes in posture and behaviour could be studied in slow motion and compared with the 'prototypical' initial ones. The EEG showed predominant rare left-sided fronto-temporal discharges. Clonazepam was changed to carbamazepin with marked improvement in behaviour, language and cognition which has been sustained up to the last control at 51 months. Videotaped home observations allowed the documentation of striking qualitative and quantitative variations in social interaction and play of autistic type in relation to the epileptic activity. We conclude that this child has a special characteristic epileptic syndrome with subtle motor and vegetative symptomatology associated with an insidious catastrophic 'autistic-like' regression which could be overlooked. The methods used to document such fluctuating epileptic behavioural manifestations are discussed.