Impact of therapeutic education on the evolution of social representations of medication in patients with Parkinson's disease: A quantitative and qualitative study (ETPARK REMED).

BACKGROUND: Among the workshops of our therapeutic patient education (TPE) program, the medication workshop (TPEM workshop) is very frequently proposed to patients in view of the difficulties they encounter related to the complexity of managing antiparkinsonian treatment. Patients' appropriation of their medications could depend on their social representations. OBJECTIVES: To evaluate the effect of our TPEM workshop on the social representations PD patients have of their medications and to compare it with that of another therapeutic intervention such as a talking group defined as the control group. METHODS: This single-center, prospective, randomized, parallel-group study investigated the social representations of medication through a questionnaire on knowledge about antiparkinsonian medications, a questionnaire on beliefs about medication (BMQ), and a word association task. RESULTS: In the TPEM group (n=16), the workshop induced significant effects over time on the knowledge questionnaire (P=0.01), BMQ specific necessity and concerns scores (P=0.04 and 0.01, respectively), necessity-concerns differential (P=0.04), and BMQ general harm (P=0.04). No significant difference was found in the talking group (n=6). Comparison of the two groups showed a significant difference of the BMQ general harm with a decrease in belief in the harmfulness of the medications in the workshop group (P=0.03). The results of the verbal association task showed a modification in the content and structure of the social representations of medication in the TPEM group. DISCUSSION: The TPEM workshop helped reduce initial negative aspects of medication representations. Improved knowledge of their medication allowed patients to feel more competent and legitimate in communicating with caregivers, modifying their beliefs about medications. Indeed, the medication was perceived as less restrictive, care becoming central as shown by the emergence of the medical team in the social representations of the medication. CONCLUSION: All the results show a specific beneficial effect of the TPEM workshop through an evolution of the social representations of medications, which became more positive in our PD patients.

[The BSE2 scale : A new clinical tool for the diagnostic of ASD within NDDs]. / L'échelle ECA2 : un nouvel outil clinique pour le diagnostic du TSA au sein des TND.

OBJECTIVES: The behavioral summarized evaluation scales, the BSE and its revised version the BSE-R, were developed and validated in the 1980-1990s. The BSE-R is still used daily by clinical teams in France and foreign countries, and it is recommended by the French Health Authority (2018). Having taken into account knowledge improvement in neurodevelopment and autism spectrum disorder (ASD) and the importance of observation by relatives in ecological context, the second version of the BSE was developed. This paper presents the construction and the validation study of the second version of the behavioral summarized evaluation scale, the BSE2 and the BSE2-P rated by parents. METHODS: Construct validity of the BSE2 scale has been studied in a population of 244 children and adolescents with ASD according to DSM-5 criteria, aged from 30 months to 18 years. Discriminant validity has been analyzed using a population of 86 patients of the same age, with neurodevelopmental disorder (NDD) without comorbidity of ASD. RESULTS: BSE2 comprises 30 items and is a two-dimensional scale as was BSE-R. Both dimensions, labelled "Interaction" (11 items) and "Modulation" (11 items), accounted for 41.7 % of the total variance. They describe autism severity and are in accordance with the two DSM-5 dimensions. Internal consistency (0.927 and 0.850 respectively) and inter-rater reliability (0.932 and 0.897 respectively) are good or excellent for both dimensions. Sensibility and specificity (0.758 and 0.767 respectively) range BSE2 among the tools with good psychometric properties. The parent version, BSE2-P, dedicated to ecological context is easily rated by parents. CONCLUSIONS: BSE2 scale for children and adolescents is a clinical tool with good psychometric properties. Its two-dimensional structure is in accordance with DSM-5 criteria. This scale covers all spectrum of ASD clinical forms in both children and adolescents. It can be used to identify ASD in complex neurodevelopmental disorders with several comorbidities and can help to distinguish autism symptomatology from other neurodevelopmental diagnoses. Furthermore, this scale allows to expand the rating context, involving parents to define and adjust the individualized therapeutic project. Thus the BSE2 is a valuable clinical tool for practitioners for both diagnosis and follow-up.

Body mass index variations in patients with Parkinson's disease treated with levodopa-carbidopa intestinal gel infusion: A case control study versus standard of care and subthalamic nucleus deep brain stimulation.

BACKGROUND: Levodopa-carbidopa intestinal gel (LCIG) is an advanced therapy for patients with Parkinson Disease (PD). Weight loss has been pointed out as an adverse event of LCIG infusion. AIMS OF THE STUDY: To compare weight changes between three groups of PD patients: patients treated with LCIG, patients within the first year of subthalamic deep brain stimulation (STN-DBS) and patients treated exclusively with oral treatment during 1 year of follow up. METHODS: Patients treated with LCIG were retrospectively matched by age, gender, disease duration and Hoehn and Yahr to patients undergoing STN-DBS and to patients both receiving the standard of care treatment and unwilling advanced therapies (SOC). Clinical features and weight were collected at baseline, and 12 months after introducing the treatment (LCIG and STN-DBS groups) or for one year of treatment (SOC). RESULTS: Eighteen patients were included in each group. They had no differences in clinical and demographic features, except for cognitive impairment. There was a mean weight (-5.8kg ±6.8) and BMI (-2.1kg/m2±2.6) reduction in the LCIG group after 12 months, while there was a slight weight loss in the SOC (-1.4kg ±3.1) and a weight increase in the STN-DBS group (5.4kg ±4.7). Differences of weight were statistically different between, LCIG and STN-DBS (P<0.001), LCIG and SOC (P=0.002) and STN-DBS and SOC (P<0.001). CONCLUSIONS: The study shows a significant weight reduction after starting LCIG infusion compared to the other groups. Weight loss should be closely monitored in patients treated with LCIG.

A new pharmacokinetic model for 90Y-ibritumomab tiuxetan based on 3-dimensional dosimetry.

Monoclonal antibodies (mAbs) are key components in several therapies for cancer and inflammatory diseases but current knowledge of their clinical pharmacokinetics and distribution in human tissues remains incomplete. Consequently, optimal dosing and scheduling in clinics are affected. With sequential radiolabeled mAb-based imaging, radiation dosing in tissues/organs can be calculated to provide a better assessment of mAb concentrations in tissues. This is the first pharmacokinetic model of 90Y-Ibritumomab tiuxetan (90Y-IT) in humans to be described, based on three-dimensional (3D) dosimetry using single-photon emission computed-tomography coupled with computed-tomography. 19 patients with follicular lymphoma were treated initially with 90Y-IT in the FIZZ trial. Based on a compartmental approach individualising the vascular compartment within studied organs, this study proposes a reliable pharmacokinetic (PK) five-compartment model replacing the currently used two-compartment model and constitutes a new direction for further research. This model provides exchange constants between the different tissues, Area Under the Curve of 111In-IT in blood (AUC) and Mean Residence Time (MRT) that have not been reported so far for IT. Finally, the elimination process appears to occur in a compartment other than the liver or the spleen and suggests the metabolism of mAbs may take place mainly on the vascular compartment level.

Factors Generating Glucose Degradation Products In Sterile Glucose Solutions For Infusion: Statistical Relevance Determination Of Their Impacts.

Sterilising glucose solutions by heat promotes the generation of a large number of glucose degradation products (GDPs). It has been shown that high levels of GDPs may result in Advanced Glycation End products that have an impact on cellular homeostasis and health in general. If data is available for peritoneal dialysis solutions, little has been published for glucose infusion fluids. It is essential to identify the parameters causing the formation of GDPs and so limit the risk of exposing patients to them. After quantifying both 5-hydroxymethyl-2-furfural, considered as an important indicator of degradation, and 2-furaldehyde, an ultimate GDP of one degradation pathway, in marketed solutions, the aim of this work is to build a model integrating all the parameters involved in the formation rates of these two GDPs: supplier, glucose amount, container material, oxygen permeability coefficient and time-lapse since manufacture. Our results show a good logarithmic relationship between GDP formation rates and time-lapse since manufacture for both GDPs. The amount of GDPs in the glucose solutions for infusion depends on the initial glucose amount, the polymer of the container, the time elapsed since manufacturing and the supplier.

Analysis of plasticizers in PVC medical devices: Performance comparison of eight analytical methods.

A wide variety of medical devices (MDs) used in hospitals are made of flexible plasticized polyvinylchloride (PVC). Different plasticizers are present in variable amounts in the PVC matrix of the devices and can leach out into the infused solutions and may enter into contact with the patients. The ARMED1 project aims to assess the migration of these plasticizers from medical devices and therefore the level of exposure in patients. For the first task of the project, eight methods were developed to directly detect and quantify the plasticizers in the PVC matrix of the MDs. We compared the overall performances of the analytical methods using standardized and validated criteria in order to provide the scientific community with the guidance and the technical specifications of each method for the intended application. We have shown that routine rapid screening could be performed directly on the MDs using the FTIR technique, with cost-effective analyses. LC techniques may also be used, but with limits and only with individual quantification of the main plasticizers expected in the PVC matrix. GC techniques, especially GC-MS, are both more specific and more sensitive than other techniques. NMR is a robust and specific technique to precisely discriminate all plasticizers in a MD but is limited by its cost and its low ability to detect and quantify plasticizer contamination, e.g. by DEHP. All these results have been confirmed by a real test, called the " blind test " carried out on 10 MD samples.

[Validation in Arabic of the Revised Autistic Behavior Summarized Evaluation Scale (BSE-R)]. / Validation en arabe de l'Échelle d'Évaluation des Comportements Autistiques, version Révisée (ECA-R).

The Behavioral Summarized Evaluation is a rating scale specifically related to the assessment of autistic behaviors in children and young teenagers with Autism Spectrum Disorder, such as communication, social interaction and interests. It also facilitates progressive recording of the evolution of children with ASD involved in short-term therapeutical studies. In the absence of standardized tools to assess autistic behaviors and effects of therapeutics in Lebanon, the BSE-R validation is an urgent need. The purpose of this study is to validate the BSE-R to the Lebanese population. METHOD: Specialized translators and clinicians ensured forward and backward translation of the scale into Arabic. The participants consisted of 100 children with ASD, diagnosed upon DSMIV-TR criterion (age range: 35 to 153 months, DS: 28.0). Fifty-eight had intellectual disability (ID). Scores of autism severity as measured by CARS1 (Schopler et al., 1980) varied from 20-58.5. Trained raters on recorded individual and group situations performed BSE-R ratings. RESULTS: BSE-R for Lebanese was found to have excellent inter-rater reliability. The internal validity highlighted a main factor which describes the severity of core autistic behaviors related to social interactions and communication, and behavioral specificities and particular interests with an internal consistency of 0.91 in a one-to-one setting and 0.92 in group settings. External validity of the scale as measured by correlations with CARS and the age of the children showed that this main factor is essentially determined by ASD severity, not by the severity of ID. It was named "relational deficiency" according to the initial paper of Barthélémy et al. (1997). CONCLUSION: BSE-R in Arabic is a practical tool, useful to all team members working with ASD children in Lebanon and the Arab countries. It will also allow future research based on reliable tools at an international level.

GABA/Glutamate synaptic pathways targeted by integrative genomic and electrophysiological explorations distinguish autism from intellectual disability.

Phenotypic and genetic heterogeneity is predominant in autism spectrum disorders (ASD), for which the molecular and pathophysiological bases are still unclear. Significant comorbidity and genetic overlap between ASD and other neurodevelopmental disorders are also well established. However, little is understood regarding the frequent observation of a wide phenotypic spectrum associated with deleterious mutations affecting a single gene even within multiplex families. We performed a clinical, neurophysiological (in vivo electroencephalography-auditory-evoked related potentials) and genetic (whole-exome sequencing) follow-up analysis of two families with known deleterious NLGN4X gene mutations (either truncating or overexpressing) present in individuals with ASD and/or with intellectual disability (ID). Complete phenotypic evaluation of the pedigrees in the ASD individuals showed common specific autistic behavioural features and neurophysiological patterns (abnormal MisMatch Negativity in response to auditory change) that were absent in healthy parents as well as in family members with isolated ID. Whole-exome sequencing in ASD patients from each family identified a second rare inherited genetic variant, affecting either the GLRB or the ANK3 genes encoding NLGN4X interacting proteins expressed in inhibitory or in excitatory synapses, respectively. The GRLB and ANK3 mutations were absent in relatives with ID as well as in control databases. In summary, our findings provide evidence of a double-hit genetic model focused on excitatory/inhibitory synapses in ASD, that is not found in isolated ID, associated with an atypical in vivo neurophysiological pattern linked to predictive coding.

Assessment of anti-factor Xa activity of heparin in binary parenteral nutrition admixtures for premature neonates.

An in vitro study was carried out to determine the anti-Xa activity of heparin in binary parenteral nutrition (BPN) admixtures for premature neonates in our neonatal intensive care unit (NICU) after a 24-hour infusion, as well as to assess drug interaction with a 50% glucose solution. Two types of bags were prepared: (1) BPN admixtures (composition defined in the NICU) including sodium heparin at 77 UI/mL and (2) bags containing only G50% with sodium heparin at 193 UI/mL. The anti-Xa activity of heparin was measured in bags at T0, after the 24-hour infusion and in eluates at the outlet of the infusion line after 24hours, using a validated chromogenic anti-Xa method. Comparisons of the mean concentration observed with the theoretical value for anti-Xa activity were performed with the Student t-test. Mean values of anti-Xa activity do not differ significantly from the values expected for all conditions. We found a slight variation in anti-Xa activity when infused over 24hours for both types of bags, with and without in-line filtration, showing that heparin remains stable during this infusion period in both BPN admixtures and G50%.

Prediction of Response to Temozolomide in Low-Grade Glioma Patients Based on Tumor Size Dynamics and Genetic Characteristics.

Both molecular profiling of tumors and longitudinal tumor size data modeling are relevant strategies to predict cancer patients' response to treatment. Herein we propose a model of tumor growth inhibition integrating a tumor's genetic characteristics (p53 mutation and 1p/19q codeletion) that successfully describes the time course of tumor size in patients with low-grade gliomas treated with first-line temozolomide chemotherapy. The model captures potential tumor progression under chemotherapy by accounting for the emergence of tissue resistance to treatment following prolonged exposure to temozolomide. Using information on individual tumors' genetic characteristics, in addition to early tumor size measurements, the model was able to predict the duration and magnitude of response, especially in those patients in whom repeated assessment of tumor response was obtained during the first 3 months of treatment. Combining longitudinal tumor size quantitative modeling with a tumor''s genetic characterization appears as a promising strategy to personalize treatments in patients with low-grade gliomas.

[Theoretical and practical aspects of cognitive remediation in intellectual disabilities: Relevance of the Cognitive Remediation Therapy program (CRT)]. / Aspects théoriques et pratiques de la remédiation cognitive dans la déficience intellectuelle du jeune adulte : La « Cognitive Remediation Therapy ¼ (CRT) peut-elle être adaptée ?

INTRODUCTION: Teenagers and adults with intellectual disabilities are nowadays "over-handicapped", often due to lack of care in self-sufficiency and continued learning, two essential domains for living in a community. Their cognitive limits, particularly on the executive functions, could be an obstacle to their involvement in the daily life activities, through their difficulties to plan, anticipate, shift and maintain information in working memory. These high level mental functions can be taught with the CRT program (Cognitive Remediation Therapy - Wykes and Reader 2005) developed in other pathologies and providing an adaptation regarding the developmental level of the person. METHODS: Firstly, it is essential to determine cognitive developmental levels of the teenager or the adult, using standard tools, such as Wechsler scales. Secondly, functional and/or adaptative levels have to be assessed using specific tools, such as the Vineland Adaptative Behavior Scale 2nd Edition (VABS-II, Sparrow et al., 2005) and the Functional Intervention Scale (EFI, Willaye et al., 2005). Finally, in order to clearly distinguish what are the preserved and impaired cognitive domains, standard tools assessing executive functions such as the Wisconsin Card Sorting Test, the Tower of London, Stroop Test and BADS are used if possible for the patient. The setting of cognitive remediation programs, previously developed for schizophrenic patients, requires adaptation for teenagers and adults with intellectual disabilities, taking into account the limitation of their cognitive abilities. In this paper, we will show that the CRT method for cognitive remediation is particularly relevant for subjects with intellectual disabilities. This method is hence focused on strategies and exercises to improve working memory, categorization and moreover executive functions. Of course this method might need adaptations, with examples based on simplification of the different tasks, notably for verbal materials, and with variations of the media used. These sessions will be part of a wider individualized caring project, allowing the person to transfer the cognitive acquisitions to his/her daily life. CONCLUSION: The use of cognitive programs adapted to people with intellectual disabilities can provide benefits in the development of autonomy and daily life activities, leading to a better quality of life and self-esteem.

An in vitro evaluation of infusion methods using a syringe pump to improve noradrenaline administration.

BACKGROUND: International guidelines recommend noradrenaline (NA) as the vasopressor of choice to treat septic shock. The aim of this study was to determine the best way to infuse patients with NA. METHODS: The in vitro study was designed to measure NA concentration at the end of each studied assembly line. Three infusion systems used the double pump method and three single pumps, which differed as regards NA concentrations (0,2 - 0,5 - 1 mg/h), dead space volume of the devices and the use of saline. Infusion systems were compared according to the time necessary to reach an NA mass flow rate steady-state plateau after the onset of infusion or after a flow change. RESULTS: Times were significantly different between the six methods for infusing NA. The system using the double syringe method with a standard extension set was the longest to reach the steady state after the onset of infusion [40.00 min (19.57 - 49.22)]. The steady-state plateau was obtained most rapidly with the double-syringe pump systems using very low dead-space volume extension sets and single-syringe pump systems containing diluted noradrenaline at the beginning of NA infusion. CONCLUSION: A combination of a low dead-space volume extension set and a double pump method with a constant saline flow rate at 5 ml/h might be the solution to provide the most reliable NA infusion delivery.

Identification of Nine New RAI1-Truncating Mutations in Smith-Magenis Syndrome Patients without 17p11.2 Deletions.

Smith-Magenis syndrome (SMS) is an intellectual disability syndrome with sleep disturbance, self-injurious behaviors and dysmorphic features. It is estimated to occur in 1/25,000 births, and in 90% of cases it is associated with interstitial deletions of chromosome 17p11.2. RAI1 (retinoic acid induced 1; OMIM 607642) mutations are the second most frequent molecular etiology, with this gene being located in the SMS locus at 17p11.2. Here, we report 9 new RAI1-truncating mutations in nonrelated individuals referred for molecular analysis due to a possible SMS diagnosis. None of these patients carried a 17p11.2 deletion. The 9 mutations include 2 nonsense mutations and 7 heterozygous frameshift mutations leading to protein truncation. All mutations map in exon 3 of RAI1 which codes for more than 98% of the protein. RAI1 regulates gene transcription, and its targets are themselves involved in transcriptional regulation, cell growth and cell cycle regulation, bone and skeletal development, lipid and glucide metabolisms, neurological development, behavioral functions, and circadian activity. We report the clinical features of the patients carrying these deleterious mutations in comparison with those of patients carrying 17p11.2 deletions.

Impact of infusion set characteristics on the accuracy of patient-controlled morphine administration: a controlled in-vitro study.

The aim of our in-vitro study was to assess the impact of infusion set characteristics on the accuracy of morphine doses in patient-controlled analgesia. Two infusion sets differing in conception and dead-space volume were assessed: a standard set and a low dead-space volume Y-set. The patient-controlled analgesia programme parameters were as follows: bolus equal to 1 ml at 100 ml.h(-1) ; lockout intervals equal to 5 and 10 min; and carrier fluid flow rate equal to 10 and 50 ml.h(-1) . Morphine concentration was determined by an ultraviolet spectrophotometric method. The morphine doses were significantly different from one set to the other during bolus and lockout intervals, whatever the patient-controlled analgesia programme. The average doses were approximately 1.3-6.0 times higher with the low dead-space volume Y-set during bolus. Our study underlines the impact of infusion set characteristics on the accuracy of morphine patient-controlled analgesia doses.

Impact of noradrenaline infusion set on mean arterial pressure: a retrospective clinical study.

OBJECTIVES: Noradrenaline (NA) can be infused through various systems including single or double syringe pumps. The aim of this study was to define the best and most efficient infusion system in an emergency context. STUDY DESIGN: This was a retrospective clinical study based on the analysis of patients' hemodynamic data. PATIENTS AND METHOD: Three infusion lines used presently in our postoperative ICU were compared through a retrospective clinical study: an NA syringe pump at 2mL/h and a saline carrier solution syringe pump at 8mL/h (infusion system 1- IS1) or 5mL/h (IS2), both connected to a very low dead-space volume set (V=0.046mL); IS3 with the same NA syringe at 2mL/h directly connected to the central venous catheter. Mean arterial pressure (MAP) was obtained from retrospective data analysis of ICU patients with postoperative septic shock criteria. Infusion systems were compared according to the time required to reach an MAP greater than 65mmHg after the onset of infusion. RESULTS: Data from 37 patients was analysed. The MAP objective was attained in 14:00 minutes (9:20 - 26:10, n=15) with IS1, in 19:10 minutes (12:20 - 27:20, n=13) with IS2 and in 34:10 minutes (23:10 - 62:30, n=9) with IS3 (P=0.00032). CONCLUSION: The use of a double syringe pump system associated with a very low dead-space volume infusion set appears to be the most appropriate system for NA infusion.

Prevention of drug delivery disturbances during continuous intravenous infusion: an in vitro study on a new multi-lumen infusion access device.

OBJECTIVE: Stopping and restarting carrier fluid flow and performing simultaneous drug infusions can lead to hazardous disturbances in drug delivery. The present study was designed to assess in vitro whether using a multi-lumen infusion access device could prevent noradrenaline disturbances. STUDY DESIGN: In vitro laboratory work. METHODS: Two infusion devices were studied: a standard device with a four-port manifold and a 150cm extension line and a nine-lumen infusion device (Edelvaiss-Multiline(®)) with eight accesses connected to nine separate lumens in a single tube of 150cm: seven accesses connected to seven peripheral lumens and one for the carrier fluid access connected to two lumens. Two experimental protocols of noradrenaline infusion were made: (a) drug flow rate change and (b) stop-and-go carrier fluid flows. Two parameters were studied: drug mass flow rate and flow change efficiency (FCE) calculated from the ratio of the area under the experimental mass flow rate curve to the area under the theoretical instantaneous mass flow rate curve. RESULTS: Variations in noradrenaline mass flow rate were more rapid with the Edelvaiss-Multiline(®) when the noradrenaline infusion rate was increased or decreased. FCE was significantly different from one infusion device to the other during both noradrenaline flow rate increase (standard vs. nine-lumen: 58% vs. 84%; P=0.008) and decrease (175% vs. 108%; P=0.008). Decreased drug delivery after stopping carrier fluid flow (standard vs. nine-lumen: 21% vs. 98%; P=0.008) and sudden temporary increases on resumption (253% vs. 103%; P=0.008) were reduced in magnitude and duration when using the Edelvaiss-Multiline(®) with a significant difference in FCE between the two infusion devices. CONCLUSIONS: Using the nine-lumen infusion device reduces drug delivery disturbances during continuous intravenous infusion.

Clinical outcome after insemination with donor sperm in patients with poor results in ICSI cycles.

The introduction of intracytoplasmic sperm injection (ICSI) provided an effective treatment for infertile couples whose infertility was attributed to male factors. However, some of them face poor results after ICSI and subsequently use artificial insemination with donor sperm (AID). Only a few studies have reported on the clinical outcome of AID cycles after previous failed ICSI cycles, with contrasting results. The results reported here involve a cohort of 47 couples undertaking 175 AID cycles after 120 failed ICSI cycles for various reasons. Couples were allocated to two groups according to the availability of top quality embryos (TQE) in ICSI cycles. In our series, AID was successful for couples with and without TQE previously transferred in ICSI cycles, the live birth rate (LBR) per cycle being 20.0% and 13.3%, respectively. However, couples with TQE tended to succeed more rapidly than couples with poor quality embryos, with a higher cumulative LBR (68.0% versus 54.5%, respectively). These findings demonstrate that even couples with a history of unsuccessful ICSI cycles because of poor embryo quality are able to achieve high LBR after AID cycles. However, such couples have a lower cumulative LBR and are required to be more patient to achieve parenthood.

Semen variation in a population of fertile donors: evaluation in a French centre over a 34-year period.

Although it has been suspected that there is a decrease in semen quality over time, the results reported to date remain debatable because of methodological issues. The aim of the study reported here was to investigate the evolution of semen quality over time in a population of 1114 fertile candidates for sperm donation at CECOS, Tours, between 1976 and 2009. We investigated semen volume, sperm concentration, progressive motility, vitality, percentage of normal forms and multiple abnormalities index of the first ejaculate in this population. We did not find a decline in semen volume, whereas we observed a significant decrease in total sperm count (from 443.2 million in 1976 to 300.2 million in 2009), motility (from 64% in 1976 to 49% in 2009) and vitality (from 88% to 80%). Moreover, a significant decline in the percentage of normal forms was noted between 1976 and 1997 (from 67% to 26%) with a steady rise in the multiple abnormalities index between 1998 and 2009 (from 1.19 to 1.65). This study involving a population of fertile men from a restricted area revealed various degrees of decline in semen parameters over a period of 34 years. These findings will have to be compared with findings in other geographical areas.

[Atypical perception processing and facial emotion disorder in autism]. / Les anomalies du traitement des émotions faciales dans l'autisme : un trouble de la perception globale.

INTRODUCTION: Autistic syndrome is defined by several abnormalities, mainly affecting social interaction skills. Disorders of the processes of processing facial and emotional stimuli, and particularly avoidance of gaze, have also been reported in this disorder. Some authors have suggested that these abnormalities may be explained, or at least contributed to, by the social disorder observed in this syndrome. The aim of this study was therefore to improve the understanding of the processes involved in perception AND the representation of faces expressing emotion in subjects with autism spectrum disorders (ASDs). METHODS: Eleven children with ASDs (mean developmental age 7 years 11 months) and eleven normally developing children (mean age 7 years 9 months) took part in three experiments. The first involved overall discrimination of emotions using photographs of faces expressing six basic emotions, the second required local emotional discrimination on the basis of isolated elements of the face (photographs of eyes and mouths isolated from the rest of the face), and for the third the children were asked to create faces expressing emotions by means of a jig-saw puzzle format, using photographs of isolated elements of the face (overall representation necessitating local discrimination). RESULTS: Our findings revealed that the normally developing children had difficulties with the process of local discrimination of emotions: their performance improved when overall perception was possible. In contrast, and astonishingly, the children with ASD were more able to discriminate isolated eyes expressing emotion than the controls, but their performance declined when overall processing was required. DISCUSSION: Our results suggested that the emotional disorders observed in ASDs might be explained by greater skills in the processing of local information. This might explain the inability of children with ASDs to achieve coherent perception of their social environment and might also lead to the withdrawal that is characteristic of this disorder. These results also suggest that the gaze avoidance that is characteristic of individuals with ASDs is eliminated when eyes are presented alone. This gaze avoidance therefore seems to be related to the complexity and variability of this type of stimulus and not to the social nature of the stimulus.