Impaired peripheral reaching and on-line corrections in patient DF: optic ataxia with visual form agnosia

An influential model of vision suggests the presence of two visual streams within the brain: a dorsal occipito-parietal stream which mediates action and a ventral occipito-temporal stream which mediates perception. One of the cornerstones of this model is DF, a patient with visual form agnosia following bilateral ventral stream lesions. Despite her inability to identify and distinguish visual stimuli, DF can still use visual information to control her hand actions towards these stimuli. These observations have been widely interpreted as demonstrating a double dissociation from optic ataxia, a condition observed after bilateral dorsal stream damage in which patients are unable to act towards objects that they can recognize. In Experiment 1, we investigated how patient DF performed on the classical diagnostic task for optic ataxia, reaching in central and peripheral vision. We replicated recent findings that DF is remarkably inaccurate when reaching to peripheral targets, but not when reaching in free vision. In addition we present new evidence that her peripheral reaching errors follow the optic ataxia pattern increasing with target eccentricity and being biased towards fixation. In Experiments 2 and 3, for the first time we examined DF’s on-line control of reaching using a double-step paradigm in fixation-controlled and free-vision versions of the task. DF was impaired when performing fast on-line corrections on all conditions tested, similarly to optic ataxia patients. Our findings question the long-standing assumption that DF’s dorsal visual stream is functionally intact and that her on-line visuomotor control is spared. In contrast, in addition to visual form agnosia, DF also has visuomotor symptoms of optic ataxia which are most likely explained by bilateral damage to the superior parietal occipital cortex. We thus conclude that patient DF can no longer be considered as an appropriate single-case model for testing the neural basis of perception and action dissociations

MxCuBE: a synchrotron beamline control environment customized for macromolecular crystallography experiments

MxCuBE is a beamline control environment optimized for the needs of macromolecular crystallography. This paper describes the design of the software and the features that MxCuBE currently provides

Identification of genetic variants associated with Huntington's disease progression: a genome-wide association study

Background Huntington's disease is caused by a CAG repeat expansion in the huntingtin gene, HTT. Age at onset has been used as a quantitative phenotype in genetic analysis looking for Huntington's disease modifiers, but is hard to define and not always available. Therefore, we aimed to generate a novel measure of disease progression and to identify genetic markers associated with this progression measure. Methods We generated a progression score on the basis of principal component analysis of prospectively acquired longitudinal changes in motor, cognitive, and imaging measures in the 218 indivduals in the TRACK-HD cohort of Huntington's disease gene mutation carriers (data collected 2008–11). We generated a parallel progression score using data from 1773 previously genotyped participants from the European Huntington's Disease Network REGISTRY study of Huntington's disease mutation carriers (data collected 2003–13). We did a genome-wide association analyses in terms of progression for 216 TRACK-HD participants and 1773 REGISTRY participants, then a meta-analysis of these results was undertaken. Findings Longitudinal motor, cognitive, and imaging scores were correlated with each other in TRACK-HD participants, justifying use of a single, cross-domain measure of disease progression in both studies. The TRACK-HD and REGISTRY progression measures were correlated with each other (r=0·674), and with age at onset (TRACK-HD, r=0·315; REGISTRY, r=0·234). The meta-analysis of progression in TRACK-HD and REGISTRY gave a genome-wide significant signal (p=1·12 × 10−10) on chromosome 5 spanning three genes: MSH3, DHFR, and MTRNR2L2. The genes in this locus were associated with progression in TRACK-HD (MSH3 p=2·94 × 10−8 DHFR p=8·37 × 10−7 MTRNR2L2 p=2·15 × 10−9) and to a lesser extent in REGISTRY (MSH3 p=9·36 × 10−4 DHFR p=8·45 × 10−4 MTRNR2L2 p=1·20 × 10−3). The lead single nucleotide polymorphism (SNP) in TRACK-HD (rs557874766) was genome-wide significant in the meta-analysis (p=1·58 × 10−8), and encodes an aminoacid change (Pro67Ala) in MSH3. In TRACK-HD, each copy of the minor allele at this SNP was associated with a 0·4 units per year (95% CI 0·16–0·66) reduction in the rate of change of the Unified Huntington's Disease Rating Scale (UHDRS) Total Motor Score, and a reduction of 0·12 units per year (95% CI 0·06–0·18) in the rate of change of UHDRS Total Functional Capacity score. These associations remained significant after adjusting for age of onset. Interpretation The multidomain progression measure in TRACK-HD was associated with a functional variant that was genome-wide significant in our meta-analysis. The association in only 216 participants implies that the progression measure is a sensitive reflection of disease burden, that the effect size at this locus is large, or both. Knockout of Msh3 reduces somatic expansion in Huntington's disease mouse models, suggesting this mechanism as an area for future therapeutic investigation

État des lieux des mesures de la satisfaction au travail en français : propositions et perspectives

International audienc

État des lieux des mesures de la satisfaction au travail en français : propositions et perspectives

International audienc

État des lieux des mesures de la satisfaction au travail en français : propositions et perspectives

International audienc

Monetary valuation of ecosystem services. An example through a water policy implementation in France

In the context of the Water Framework Directive, the French Water Agencies were asked to justify exemptions, for disproportionate cost reasons, to the “good water” status in a series of river basins in 2015. The ministry of the Environment and the economists of the Water Agencies agreed on the fact that this justification would be based on cost-benefit analyses. A great number of cost-benefit analyses were carried out on water bodies or groups of water bodies. This article reports on this experience conducted by the French Water Agencies. It highlights the fact that the large majority of restoration projects generated higher costs than benefits. It suggests that the implementation of the Water Framework Directive could be considerably limited by the use of the Costs-Benefits-Analysis. This result is discussed, especially with regard to the assessment of non-market benefits. These methods are in fact a source of much technical uncertainty which can be used in a strategic way to justify lack of implementation of the EU Directive. Last, the article questions the limits of economic referentials against the legal referential in the implementation of environmental policy.L’État a sollicité les agences de l’eau françaises pour justifier des dérogations à l’atteinte du « bon état » des eaux en 2015, pour cause de coûts disproportionnés, sur un certain nombre de petits bassins versants. En accord avec le ministère chargé de l’environnement, il a été convenu de s’appuyer pour ce faire sur des analyses coûts-bénéfices. Le présent article rend compte de cette expérience et des questionnements suscités par le chiffrage des bénéfices non marchands, qui se sont posés en termes techniques, stratégiques et éthiques, pour pointer plus globalement les limites de l’usage des référentiels économiques dans le cadre de l’application d’une politique environnementale

The use of cost–benefit analysis in environmental policies: Some issues raised by the Water Framework Directive implementation in France

International audienceThis paper aims to practically contribute to the literature on the use of cost–benefit analysis (CBA) and economic evaluation in environmental decision-making through a practical case study: the implementation of the Water Framework Directive (WFD) in France, for the first cycle (2010–2015). The WFD requires that Member States achieve “good status” for all water bodies in 2015. However, exemptions can apply, if justified, on natural, technical or economic reasons. For the latter, EU guidance documents recommend to use CBA. In France, the water agencies carried out 710 CBAs on proposed restoration projects for water bodies. This article reports on this experience. Issues concerning these analyses are discussed, especially the assessment of non-market benefits. Finally, this article questions the use of economic analysis in the implementation of environmental policy