'Emotiplay': a serious game for learning about emotions in children with autism: results of a cross-cultural evaluation.

Children with autism spectrum conditions (ASC) experience difficulties recognizing others' emotions and mental states. It has been shown that serious games (SG) can produce simplified versions of the socio-emotional world. The current study performed a cross-cultural evaluation (in the UK, Israel and Sweden) of Emotiplay's SG, a system aimed to teach emotion recognition (ER) to children with ASC in an entertaining, and intrinsically motivating way. Participants were 6-9 year olds with high functioning ASC who used the SG for 8-12 weeks. Measures included face, voice, body, and integrative ER tasks, as well as parent-reported level of autism symptoms, and adaptive socialization. In the UK, 15 children were tested before and after using the SG. In Israel (n = 38) and Sweden (n = 36), children were randomized into a SG or a waiting list control group. In the UK, results revealed that 8 weeks of SG use significantly improved participants' performance on ER body language and integrative tasks. Parents also reported their children improved their adaptive socialization. In Israel and Sweden, participants using the SG improved significantly more than controls on all ER measures. In addition, parents in the Israeli SG group reported their children showed reduced autism symptoms after using the SG. In conclusion, Emotiplay's SG is an effective and motivating psycho-educational intervention, cross-culturally teaching ER from faces, voices, body language, and their integration in context to children with high functioning ASC. Local evidence was found for more generalized gains to socialization and reduced autism symptoms.

Home administration of bortezomib in multiple myeloma is cost-effective and is preferred by patients compared with hospital administration: results of a prospective single-center study.

BACKGROUND: Subcutaneous (s.c.) administration of bortezomib is the most widely used route of administration for the treatment of patients with multiple myeloma. No study has as yet prospectively evaluated home versus hospital administration of s.c. bortezomib with respect to patient preference and cost. PATIENTS AND METHODS: In this prospective trial, myeloma patients received the first administration of s.c. bortezomib of each cycle in the outpatient unit of the Department of Hematology. When possible, all subsequent doses of bortezomib within each cycle were provided at home. A cost analysis was carried out to compare the average cost of an injection of bortezomib in the outpatient unit and at home. In order to compare hospital and home administration of bortezomib for preference and satisfaction, patients had to complete 2 simple questionnaires analyzing 16 criteria, such as quality of life, well-being, social life, satisfaction, safety, quality of care, the reduction in personal transportation time, and personal anxiety. Each item was analyzed using a Likert scale. RESULTS: Fifty patients were studied. Overall, a total of 1043 s.c. injections of bortezomib were carried out, 655 (62.8%) at home, and 388 (35.2%) in the outpatient unit. The cost analysis showed that the total cost of one s.c. injection of bortezomib in the outpatient unit was €1510.09 versus €1224.57 for the home administration, which represents a reduction of €285.52, i.e. 20% of the cost of the hospital administration. The evaluation of patient preference and satisfaction showed that home administration improved the quality of life in 84% of the patients, increased well-being in 78%, and improved the activities of daily living in 72% of the cases. Overall, 98% of the patients noted their preference for home administration over the hospital administration of bortezomib. CONCLUSION: Home administration of s.c. bortezomib is cost-effective and is preferred by myeloma patients compared with hospital administration.

Emotional contagion for pain is intact in autism spectrum disorders.

Perceiving others in pain generally leads to empathic concern, consisting of both emotional and cognitive processes. Empathy deficits have been considered as an element contributing to social difficulties in individuals with autism spectrum disorders (ASD). Here, we used functional magnetic resonance imaging and short video clips of facial expressions of people experiencing pain to examine the neural substrates underlying the spontaneous empathic response to pain in autism. Thirty-eight adolescents and adults of normal intelligence diagnosed with ASD and 35 matched controls participated in the study. In contrast to general assumptions, we found no significant differences in brain activation between ASD individuals and controls during the perception of pain experienced by others. Both groups showed similar levels of activation in areas associated with pain sharing, evidencing the presence of emotional empathy and emotional contagion in participants with autism as well as in controls. Differences between groups could be observed at a more liberal statistical threshold, and revealed increased activations in areas involved in cognitive reappraisal in ASD participants compared with controls. Scores of emotional empathy were positively correlated with brain activation in areas involved in embodiment of pain in ASD group only. Our findings show that simulation mechanisms involved in emotional empathy are preserved in high-functioning individuals with autism, and suggest that increased reappraisal may have a role in their apparent lack of caring behavior.

Fatigue and respiratory disorders.

OBJECTIVES: To analyze the factors at the origin of fatigue in respiratory disorders. To assess fatigue and its functional impact on patients affected from respiratory diseases. To evaluate the results of comprehensive care on fatigue and functional capacity. MATERIALS AND METHODS: We systematically reviewed the literature in Medline and the Cochrane Library, using the following keywords: fatigue, respiratory disorders, questionnaire, evaluation, assessment, randomized controlled trial, meta-analysis. RESULTS: Fatigue is a high frequency symptom (90%) and takes an important place, as much as dyspnea, in the genesis of the respiratory induced handicap. Its assessment is varied, according to the studies. It originates from multiple causes, as shown from clinical and experimental studies. The main treatment consists in rehabilitation, using physical exercises. Its efficacy is demonstrated on physical endurance, but is not clear in terms of general fatigue. CONCLUSION: Although fatigue is very frequent complaint, along with a major disabling condition, the comprehensive assessment of fatigue, in respiratory disorders, including its physical and cognitive components, is not still really codified. Rehabilitation is the main treatment. Its efficiency has been demonstrated on the physical and functional components of fatigue. Its results on perceived fatigue remains to be evaluated.

[Two cases of rehabilitation in Ehler-Danlos syndrome]. / Prise en en charge rééducative de la maladie d'Elhers-Danlos: à propos de deux cas.

PURPOSE: Ehlers-Danlos syndrome is rare and little known. It comprises a heterogeneous group of heritable connective tissue disorders characterized by articular hypermobility, skin extensibility and tissue fragility. Diagnosis may be difficult and often delayed. CASE REPORT: Here we describe 2 cases of women with Ehlers-Danlos syndrome. The first had scoliosis with back pain and joint hypermobility. The second presented with hypermobility and joint dislocation, which increased during pregnancy. In these 2 cases, rehabilitation management produced clinical improvement. DISCUSSION: Orthopaedic complications such joint pain, joint swelling, joint dislocation, back pain, with walking and hand function disability are the main problems in Ehlers-Danos syndrome. Surgery may be necessary to correct dislocated joints but is often not sufficient to resolve the handicap, and physical therapy has an important place in management. CONCLUSION: Ehlers-Danlos syndrome is an evolving disease that can lead to great impairment. Thus, physicians must be aware of this syndrome to offer the best management, with the appropriate use of orthotic devices, specific strengthening routines, education in proper body mechanics and assistive devices, to prevent joint dislocation and subluxations responsible for pain and handicap.

[Multiple sclerosis and physical activities]. / Activité sportive et sclérose en plaques.

INTRODUCTION: Multiple sclerosis (MS) is a neurological disease of the central nervous system that most often affects young people. It is characterized by various debilitating symptoms. The aim of this study was to assess physical activities in patients with MS and how these affect their quality of life. METHODS: Forty-five patients were questioned about their physical activities. The following were taken into account: the characteristics of MS within each patient, the level of disease severity (EDSS score) and the quality of life (on the SEP-59 questionnaire). RESULTS: Fifteen patients had to give up a physical activity because of their illness but more so due to ataxia, fatigue and muscular weakness. Fifteen patients take part in a physical activity, the principal motivation being for their personal well-being. The mean EDSS was 4.2. Answers on the SEP-59 revealed found that patients with MS who took part in a physical activity had significantly better well-being in general. DISCUSSION AND CONCLUSION: Physical activities seem to improve the quality of life of patients with MS who, in general, already have a poor quality of life in relation to the general population. Knowing that muscular function is improved through exercise in patients with an EDSS less than 6, it would seem advantageous for them to take part in a regular physical activity.

[Treatment of heterotopic ossification by extracorporeal shock wave: 26 patients]. / Traitement des paraostéoarthropathies par ondes de choc. Etude sur 26 patients.

OBJECTIVES: To evaluate the effects of extracorporeal shockwave therapy (ESWT) on heterotopic ossification leading to functional limitations in the short and medium term. METHODS: Twenty-six patients with heterotopic ossification received sessions of ESTW (4000 shocks, 3/s), with an energy ranging from 0.54 to 1.06 mJ/mm2, once a week for 4 consecutive weeks. Intermediary assessments performed 1 month after the last session related to pain (on a visual analog scale [VAS]), range of motion, functional independence (FIM), walking distance (whenever possible), radiology, and blood calcium and alkaline phosphatase levels. Eighteen patients with total hip arthroplasty (THA) were followed up by quiz, at 11 months, on average. RESULTS: Heterotopic ossification was neurogenic in 5 patients and nonneurogenic in 21. The length of evolution of ossification was 32+/-21 months. The measurements showing significant improvement in the short term were pain, with a mean decrease of 4.32 to 1.14 on a VAS; joint flexion, with an mean increase of 8.18+/-11.9 degrees; and walking distance, with a mean increase from 1126 to 2776 m. The treatment was tolerated for the most part. THA cases showed a decline in factors initially shown to be improved. However, the long-term results were superior to clinical status before treatment. CONCLUSION: ESWT might be an interesting treatment for heterotopic ossification and can be a complement to usual medical treatment, physiotherapy, and before surgery.

[Home care and quality of life three years after stroke]. / Maintien à domicile et qualité de vie à distance d'un accident vasculaire cérébral.

INTRODUCTION: Stroke is the most disabling chronic disease among adults. The assessment of quality of life is of great importance to improve the management of patients with stroke sequelae returning to the community. METHOD: A total of 93 patients and their referring clinicians were contacted. Quality of life of patients three years after stroke was assessed by use of the Short-Form Health Survey (SF-36). RESULTS: Thirteen patients died during this period. Fifty participated in the study. The average age was 66.8 years. The patients who died were older and had an initial greater impairment after stroke than survivors. In most of the cases, a life plan, built during the inpatient rehabilitation period, could be achieved. Just six patients had to change their way of life during the three years after stroke. A total of 44% of the patients needed human assistance for basic daily living activities. Patients with stroke had lower scores on the SF-36 than their healthy peers. All subscale scores differed for patients with stroke, as compared with health peers. Initial disability, the occurrence of falls, age and urinary incontinence at the time of the study indicated poor prognosis. DISCUSSION: Our results are close to those previously published: a great number of people who survive stroke live with sequelae that requires assistance in the home and have decreased quality of life. These results stress the importance of follow-up by a rehabilitation team to prevent altered quality of life, with a special care to urinary incontinence and falls, especially in old people with abundant sequelae of stroke.

[Charcot-Marie-Tooth disease associated with syringomyelia revealed by urinary disorders]. / Association de maladie de Charcot-Marie-Tooth et de syringomyélie révélée par la survenue de troubles urinaires.

PURPOSE: Charcot-Marie-Tooth (CMT) disease is characterized by genetic and clinical heterogeneity. The occurrence of urinary disorders is unusual and requires further investigation. CMT disease and syringomyelia are not usually associated. Moreover, primary urinary signs in syringomyelia are not frequent. CASE REPORT: An 11-year-old girl with CMT disease 1A duplication presented with motor deficit of the leg associated with urinary disorders. Urodynamic study revealed neurogenic detrusor overactivity, and magnetic resonance imaging confirmed syringomyelia extending from T11 to an L1 located conus. DISCUSSION: Review of the literature confirmed the possibility of primary urinary signs in syringomyelia, with only one case of CMT and urinary signs described. In this case, the diagnosis was radicullar and medullar compression by a hypertrophic nerve formation called an "onion bulb" reflecting the demyelinisation and remyelinisation cycle. CONCLUSION: Urinary signs in CMT disease are exceptional and require investigation into the possibility of a central lesion.

[Influence of temperature changes on clinical symptoms in multiple sclerosis: an epidemiologic study]. / Influence des variations de la température sur la symptomatologie clinique dans la sclérose en plaques: étude épidémiologieque.

UNLABELLED: The effects of the modifications of temperature are well known in patients affected by multiple sclerosis (MS). They are variable and can influence daily living. This sensibility can be used in the management of the disabilities. METHOD: An epidemiological study was realized on a cohort of 191 patients suffering from MS referred to the MS clinic of Rennes (France). All the patients were questioned about the influence of heat and cold on their clinical symptoms (fatigue spasticity, walking disorders, vision, em leader ). Correlations with the main clinical characteristics were studied. POPULATION: One hundred ninety-one patients, 129 women and 62 men with an average age of 47.6 +/- 10 years were interviewed. Average score EDSS was of 5.2 +/- 1.5. The mean duration of MS was 13.5 +/- 10 years. RESULTS: One hundred forty-seven patients (77%) reported a sensibility to the temperature. Heat deteriorated function in 104 cases and 82 patients improved with cold. Paradoxically 20 patients reported to be deteriorated with cold and 19 improved with heat. Fatigue and walking were the most sensitive to temperature fluctuations. No particular clinical profile could be established. Fifty percent of the patients used this sensibility with therapeutic aim in everyday life. DISCUSSION: The clear influence of temperature fluctuations on the clinical symptom was confirmed in this study. However, there is a great variability from one patient to another. Different hypotheses have been evoked to explain this phenomenon. The most likely is an influence on the nervous specific conductivity. In routine practice cold physiotherapy will be proposed on case by case basis and still has an interesting place in the rehabilitation management.

[Interest of visual biofeedback training in rehabilitation of balance after stroke]. / Intérêt de la rééducation par biofeedback visuel sur plateforme de stabilométrie dans la prise en charge des troubles posturaux des hémiplégiques vasculaires.

OBJECTIVE: The aim of this study was the assessment of visual biofeedback in the management of balance disorders after stroke. MATERIAL AND METHODS: This is a prospective randomised trial, which compares visual biofeedback training and traditional balance training. Twenty-seven patients took part in this study: 13 in the first group and 14 in the second group. The rehabilitation program in the first group consists in an association of visual biofeedback and traditional training during 3 weeks. For the second group traditional training was used alone. Clinical, functional and forceplate assessments were performed before and after the program of rehabilitation. RESULTS: All the patients had a clinical and functional improvement. However, patients who have benefited from biofeedback training had a significant improvement of the forceplate parameters with closed eyes. DISCUSSION AND CONCLUSION: Those results confirm the interest of rehabilitation in balance disorders after stroke. The use of biofeedback training incorporated into functional physiotherapy affords added benefits probably by a best integration of proprioceptive informations.

[Comparative study of respiratory mechanisms between professional lyrical singers and beginners]. / Etude comparative des stratégies respiratoires entre les chanteurs lyriques professionnels et débutants.

The apprenticeship for classical singing requires years of training due to its complexity. This training is directed at the different levels of the vocal apparatus: the source of energy or air flow (lungs), the vibrator (vocal cords) and the resonators (bucco-pharyngeal cavity). For this study we have concentrated on the first level, that is, respiration. When a greater demand of vocal activity is required respiration becomes more sustained and more complex; this is when difficulties may be observed. Singers overcome these difficulties through different strategies according to their level of vocal training. Our study compared respiratory strategies used by professional singers and singing students (first year conservatoire students) during singing tasks; none of the subjects had any history of vocal pathology. Electromyographic analysis of muscle activity was registered for the rectus abdominals muscle, external oblique muscle and transverse muscle with surface electrodes. Kinetic analysis was used to measure thoracic cage displacement: pressure sensitive belts were placed around the pubis, the epigastric region and thorax. The subjects were asked to perform two vocal tasks: vocalization of a sustained "i" and singing of an extract of a vaccaï. Results were compared between the 7 professional singers and the 6 singing students. They allowed us to confirm the existence of distinct respiratory strategies according to the level of vocal training, as described in literature. Professional singers inhibited the activity of their rectus abdominals muscles during singing and used mostly their external oblique and transverse muscles, thus, sustaining an expanded ribcage and a longer expiratory breath. Singing students, on the other hand, worked intensively all the abdominal muscles leading to a collapse of the ribcage. Management of air was more difficult and breath was shorter. Future studies will consider a larger population sample in order to define quantitative parameters that might allow a significant differentiation of respiratory strategies between professional singers and singing students.