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Background: The present study investigated the influence of emotional contexts on mental flexibility in adults with Prader–Willi syndrome (PWS) using a voluntary task‐switching paradigm that was implemented with emotionally valenced pictures. The study aims were to assess whether adults with PWS have impaired switching abilities, whether the deficit is specific to PWS or linked to intellectual disabilities, and the influence of emotional contexts on performance. Method: The task‐switching performance of 30 adults with PWS was compared with that of 30 healthy adults matched on chronological age, and to that of 30 adults with intellectual disabilities but without PWS, matched on intellectual quotient level and chronological age. Indicators of switching performance were switching cost and repetition bias. Emotional contexts were operationalised with positive, neutral and negative task‐irrelevant pictures. Results: Adults with PWS showed a large increase in switching costs compared with the two control groups, and this effect did not vary across emotional contexts. More fine‐tuned examination revealed subtle performance modulations: negative contexts tended to increase the repetition bias in all three groups while positive contexts slowed down global performance in PWS. Conclusions: The results confirmed previous studies, showing impaired switching abilities in PWS over and beyond the influence of intellectual level, but revealed no robust variations in switching deficits across emotional contexts. [ABSTRACT FROM AUTHOR]

Goal Attainment Scaling (GAS) is a method for writing person-centred approach evaluation scales that can be used as an outcome measure in clinical or research settings in rehabilitation. To be used in a research setting, it requires a high methodological quality approach. The aim of this study was to explore the feasibility and reliability of the GAS quality rating system, to ensure that GAS scales used as outcome measures are valid and reliable. Secondary objectives were: (1) to compare goal attainment scores' reliability according to how many GAS levels are described in the scale; and (2) to explore if GAS scorings are influenced by who scores goal attainment. The GAS scales analysed here were set collaboratively by 57 cognitively impaired adults clients and their occupational therapist. Goals had to be achieved within an inpatient one-month stay, during which clients participated in an intervention aimed at improving planning skills in daily life. The GAS quality rating system proved to be feasible and reliable. Regarding GAS scores, interrater reliability was higher when only three of the five GAS levels were described, i.e., "three milestone GAS" (0.74-0.92), than when all five levels were described (0.5-0.88), especially when scored by the clients (0.5 -0.88).

Interference effect of food and emotional stimuli in Stroop-like tasks for children and adults with Prader-Willi Syndrome. The aim of this work was to study the way items related to food or emotion are processed by a population known to have difficulties with dietary restriction, namely individuals with Prader-Willi Syndrome (PWS). Given the presence of intellectual disability (ID) in PWS, our experiments were designed to examine whether these difficulties were specific to PWS or linked with their ID. Two modified Stroop tasks (i.e., a food version and an emotional version) were administered to seventy-four children (aged between 6 and 16 years old) divided into three groups (one with PWS, one with ID matched on age and Intellectual Quotient (IQ), and one healthy group matched on age) and to eighty-four adults (aged between 18 and 48 years old) distributed in the same three groups. For both tasks, a picture version was used for the children and a word version for the adults. For the food Stroop task, (Experiment 1), materials were composed of low or high-caloric food items and stimuli not related to food. The results show a food Stroop effect for children and adults with PWS that was absent in the group of healthy participants. Moreover, a food Stroop effect was also significant for adults with ID. For the emotional Stroop task (Experiment 2), materials were composed of negative, positive and neutral stimuli. The emotional Stroop effect was also obtained for children and adults with PWS as well as for the healthy group, but not for the age- and IQ-matched group. For the PWS groups, results show a preservation to process positive pictures for children and difficulties to process negative stimuli for both age-groups. These results suggest that people with PWS have difficulties in disengaging their attention when food stimuli are present in their environment and poorer abilities to process negative ones. These difficulties endure in adulthood.

Sezary syndrome (SS) is a rare leukemic form of cutaneous T-cell lymphoma. Diagnosis mainly depends on flow cytometry, but results are not specific enough to be unequivocal. The difficulty in defining a single marker that could characterize Sezary cells may be the consequence of different pathological subtypes. In this study, we used multivariate flow cytometry analyses. We chose to investigate the expression of classical CD3, CD4, CD7, and CD26 and the new association of 2 markers CD158k and PD-1. We performed lymphocyte computational phenotypic analyses during diagnosis and follow-up of patients with SS to define new SS classes and improve the sensitivity of the diagnosis and the follow-up flow cytometry method. Three classes of SS, defined by different immunophenotypic profiles, CD158k+ SS, CD158k-PD-1+ SS, CD158k and PD-1 double-negative SS, showed different CD8+ and B-cell environments. Such a study could help to diagnose and define biological markers of susceptibility/resistance to treatment, including immunotherapy., (© 2022 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.)

Prader-Willi Syndrome (PWS) is a neurodevelopmental genetic disorder with executive deficits. Planning is one of the impaired executive functions implied in the regulation of behavior and everyday actions. We aimed to explore the feasibility and the effectiveness of a metacognitive strategy training designed to improve planning in adults with PWS using a double-blind between-group (training versus usual care) randomized controlled trial, with computerized tests and paper-pencil ecological outcome measures targeting planning, other executive functions, and achievement of personalized goal. Results showed better performances in several executive tasks and in achievement of personalized goals after both interventions, but better improvement for the experimental group (n = 27) compared to control (n = 26) only on the task assessing planning abilities. Interviews with occupational therapists demonstrated the feasibility of this training with this population. Despite a small number of sessions, the metacognitive strategy training showed encouraging results on planning abilities of patients.

Background: Prader-Willi syndrome (PWS) is a complex developmental genetic disorder associated with intellectual disability and deficits in executive functions which result in disorganisation and poor personal autonomy., Aims: This study aimed to determine impairments in planning skills of adults with PWS, in relation with their intellectual disabilities, as well as the influence of food compulsions on their performance., Methods and Procedures: A modified version of the Zoo Map from the Behavioural Assessment of the Dysexecutive Syndrome was used in three groups: a group of adults with PWS in comparison with two groups both matched on chronological age, one with typical development (TD) and one with intellectual disability (ID)., Outcomes and Results: Compared to TD adults, both adults with PWS and ID showed increased planning time and lower raw scores on the planning task. The execution time and the number of errors were higher in the PWS group compared to the comparison groups. All three groups performed worse in the non-food condition only for number of errors and raw score., Conclusions and Implications: Planning abilities were impaired in PWS adults. Results also showed that intellectual level plays a role in participants' performance. These findings are essential to understand the difficulties of people with PWS daily life., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Background : Prader-Willi syndrome (PWS) is a neurodevelopmental genetic disorder involving executive deficits notably with planning. The main objective of the study is to assess the effectiveness of cognitive training on daily life planning difficulties in PWS patients. Methods/design : The study is a double-blind randomized controlled trial which will compare the effectiveness of a metacognitive strategy intervention designed to improve planning difficulties for PWS patients to usual occupational therapy. Sixty adults will be included over 20 months. The main outcome measure will be the performance on the Modified Six Elements Test from the BADS; secondary outcome measures will be computerized executive tasks and questionnaires. Daily life planning difficulties will be identified and transformed into measurable goals using Goal Attainment Scaling. Discussion : The project will provide knowledge on the difficulties experienced by PWS patients, in relation to their executive functioning in order to implement effective intervention for planning in daily life.

We present a noncontact optical metrology measuring the pistons and tip/tilt angles of the 61 hexagonal segments of a compact-sized segmented mirror. The instrument has been developed within the scope of a design study for the European Extremely Large Telescope (E-ELT). It is used as reference sensor for cophasing of the mirror segments in closed-loop control. The mirror shape is also measured by different types of stellar light-based phasing cameras whose performances will be evaluated with regard to a future E-ELT. Following a description of the system architecture, the second part of the paper presents experimental results demonstrating the achieved precision: 0.48 nm rms in piston and 74 nrad rms in tip/tilt., ((c) 2008 Optical Society of America)