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This systematic review analyzes the differential use of gestures in learning by children with intellectual disability (ID) compared to typically developing ones (TD). Eleven studies published between 2000 and 2020 fulfilled the inclusion criteria ( N  = 364 participants). The results identify three key elements: (1) Children with ID accompany their spoken language with more gestures than TD children; (2) Specifically, they produce more iconic gestures that provide access to the conceptualization process and understanding in students with ID; (3) Children with ID rely on gesture more than TD children to carry meaning (i.e., produce unimodal gestural utterances without accompanying speech). Possible implications for teaching and guidelines for future research are proposed.

The gestures produced by children with intellectual disability (ID) in spatial tasks are rarely considered, although they have a supporting role in the formation of thought. In this research study, we analyzed the number of gestures, the type of gestures, and their role in the expression of knowledge of students with ID. Twenty students (12–17 years old) with ID and 40 students with typical development (TD) matched on visual-spatial level (n = 20) and on language level (n = 20) participated in the research. Students with ID made significantly more gestures in relation to the number of words spoken compared to their peers with TD. Thirty percent of the expressive communication of students with ID came from gestures alone, and 60% of the responses contained at least one gesture. Finally, the higher the level of task difficulty, the more gestures the students made.

Background: Current data show an increase in stress among youth since the COVID-19 pandemic, raising the question of the measures to be put in place to limit it. Aim: The aim of this study is to measure the prevalence of burnout and the different risk and protective factors of burnout among students and to compare the mean scores obtained with those collected in a similar sample in 2014. Method: Perceived health, school burnout, and the different risk and protective factors among students were measured by self-reported questionnaires among a sample of 184 students (11–13 years old). Results: The results indicate significantly higher burnout scores than in 2014. Different variables were predictive of school burnout: 1. At the individual level, a low academic achievement in mathematics, somatic symptoms, and the expressed stress; 2. At the interpersonal level, lack of support from peers, teachers, and parents and a negative classroom climate; 3. At the pandemic level, the increased workload related to the consequences of COVID-19. The factors with the greatest impact are confidence in the future, perceived stress, parental supports, and mathematics results. Conclusions: An intervention program targeting these four factors among burned out students would be relevant to set up in order to reduce its prevalence.

Metastatic breast cancer patients receive lifelong medication and are regularly monitored for disease progression. The aim of this work was to (1) propose networks to segment breast cancer metastatic lesions on longitudinal whole-body PET/CT and (2) extract imaging biomarkers from the segmentations and evaluate their potential to determine treatment response. Baseline and follow-up PET/CT images of 60 patients from the EPICUREseinmeta study were used to train two deep-learning models to segment breast cancer metastatic lesions: One for baseline images and one for follow-up images. From the automatic segmentations, four imaging biomarkers were computed and evaluated: SULpeak, Total Lesion Glycolysis (TLG), PET Bone Index (PBI) and PET Liver Index (PLI). The first network obtained a mean Dice score of 0.66 on baseline acquisitions. The second network obtained a mean Dice score of 0.58 on follow-up acquisitions. SULpeak, with a 32% decrease between baseline and follow-up, was the biomarker best able to assess patients’ response (sensitivity 87%, specificity 87%), followed by TLG (43% decrease, sensitivity 73%, specificity 81%) and PBI (8% decrease, sensitivity 69%, specificity 69%). Our networks constitute promising tools for the automatic segmentation of lesions in patients with metastatic breast cancer allowing treatment response assessment with several biomarkers.

Metastatic breast cancer patients receive lifelong medication and are regularly monitored for disease progression. The aim of this work was to (1) propose networks to segment breast cancer metastatic lesions on longitudinal whole-body PET/CT and (2) extract imaging biomarkers from the segmentations and evaluate their potential to determine treatment response. Baseline and follow-up PET/CT images of 60 patients from the EPICUREseinmeta study were used to train two deep-learning models to segment breast cancer metastatic lesions: One for baseline images and one for follow-up images. From the automatic segmentations, four imaging biomarkers were computed and evaluated: SULpeak, Total Lesion Glycolysis (TLG), PET Bone Index (PBI) and PET Liver Index (PLI). The first network obtained a mean Dice score of 0.66 on baseline acquisitions. The second network obtained a mean Dice score of 0.58 on follow-up acquisitions. SULpeak, with a 32% decrease between baseline and follow-up, was the biomarker best able to assess patients' response (sensitivity 87%, specificity 87%), followed by TLG (43% decrease, sensitivity 73%, specificity 81%) and PBI (8% decrease, sensitivity 69%, specificity 69%). Our networks constitute promising tools for the automatic segmentation of lesions in patients with metastatic breast cancer allowing treatment response assessment with several biomarkers.

ObjectivesVacuoles, E1 enzyme, X-linked, autoinflammatory and somatic (VEXAS) syndrome is an adult-onset autoinflammatory disease associated with somatic ubiquitin-like modifier-activating enzyme 1 (UBA1) mutations. We aimed to evaluate the efficacy and safety of targeted therapies.MethodsMulticentre retrospective study including patients with genetically proven VEXAS syndrome who had received at least one targeted therapy. Complete response (CR) was defined by a clinical remission, C-reactive protein (CRP) ≤10 mg/L and a ≤10 mg/day of prednisone-equivalent therapy, and partial response (PR) was defined by a clinical remission and a 50% reduction in CRP levels and glucocorticoid dose.Results110 patients (median age 71 (68–79) years) who received 194 targeted therapies were included: 78 (40%) received Janus kinase (JAK) inhibitors (JAKi), 51 (26%) interleukin (IL)-6 inhibitors, 33 (17%) IL-1 inhibitors, 20 (10%) tumour necrosis factor (TNFα) blockers and 12 (6%) other targeted therapies. At 3 months, the overall response (CR and PR) rate was 24% with JAKi, 32% with IL-6 inhibitors, 9% with anti-IL-1 and 0% with TNFα blockers or other targeted therapies. At 6 months, the overall response rate was 30% with JAKi and 26% with IL-6 inhibitors. Survival without treatment discontinuation was significantly longer with JAKi than with the other targeted therapies. Among patients who discontinued treatment, causes were primary failure, secondary failure, serious adverse event or death in 43%, 14%, 19% and 19%, respectively, with JAKi and 46%, 11%, 31% and 9%, respectively, with IL-6 inhibitors.ConclusionsThis study shows the benefit of JAKi and IL-6 inhibitors, whereas other therapies have lower efficacy. These results need to be confirmed in prospective trials.

Background VEXAS syndrome (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) is a recently described syndrome linked to somatic mutations in the UBA1 gene, causing systemic autoinflammatory manifestations. To date, few data are available concerning neurological manifestations. The aim of this study was to describe their prevalence, clinical spectrum and outcome under treatment. Methods Retrospective multicentre study including patients with VEXAS syndrome from the French VEXAS Registry between November 2020 and March 2023. Additional cases were included after a national call for observations. Each patient with confirmed UBA1 somatic mutation and neurological manifestation was reviewed during multidisciplinary meetings. Clinical, radiological, biological characteristics, treatments, and outcome were described. Results Of the 291 patients included in the French VEXAS Registry, 17 (6%) had central (CNS) or peripheral (PNS) neurological involvement, with 13 additional cases identified by the national call. Of the 30 patients included, 21 (70%) had PNS involvement and 9 (30%) CNS involvement. PNS involvements included polyneuropathy ( n = 9), cranial nerve involvement ( n = 7), non-length-dependent polyneuropathy ( n = 5) and multiple mononeuropathy ( n = 3). CNS involvements included encephalopathy ( n = 6), lacunar cerebral infarcts ( n = 4), posterior reversible encephalopathy syndrome ( n = 3) and optic perineuritis ( n = 2). Most neurological manifestations were improved by steroids (68%), steroid-sparing agents were used in 90% [most frequently ruxolitinib ( n = 11), azacitidine ( n = 8), tocilizumab ( n = 4)], and mortality was 30% after a median follow-up of 4 years. Conclusions Neurological manifestations may occur in a small but possibly underestimated proportion of patients with VEXAS syndrome, are heterogeneous and can involve both PNS and CNS.

Aims/hypothesis Regulation of energy balance involves the participation of many factors, including nutrients, among which are circulating lipids, acting as peripheral signals informing the central nervous system of the energy status of the organism. It has been shown that neuronal lipoprotein lipase (LPL) participates in the control of energy balance by hydrolysing lipid particles enriched in triacylglycerols. Here, we tested the hypothesis that LPL in the mediobasal hypothalamus (MBH), a well-known nucleus implicated in the regulation of metabolic homeostasis, could also contribute to the regulation of body weight and glucose homeostasis.Methods We injected an adeno-associated virus (AAV) expressing Cre-green fluorescent protein into the MBH of Lpl-floxed mice (and wild-type mice) to specifically decrease LPL activity in the MBH. In parallel, we injected an AAV overexpressing Lpl into the MBH of wild-type mice. We then studied energy homeostasis and hypothalamic ceramide content.Results The partial deletion of Lpl in the MBH in mice led to an increase in body weight compared with controls (37.72 +/- 0.7 g vs 28.46 +/- 0.12, p < 0.001) associated with a decrease in locomotor activity. These mice developed hyperinsulinaemia and glucose intolerance. This phenotypealso displayed reduced expression of Cers1 in the hypothalamus as well as decreased concentration of several C18 species of ceramides and a 3-fold decrease in total ceramide intensity. Conversely, overexpression of Lpl specifically in the MBH induced a decrease in body weight.Conclusions/interpretation Our study shows that LPL in the MBH is an important regulator of body weight and glucose homeostasis.