Progression of motor subtypes in Huntington’s disease. a 6-year follow-up study

The objective of this study is to investigate the progression of predominantly choreatic and hypokinetic-rigid signs in Huntington's disease (HD) and their relationship with cognitive and general functioning over time. The motor signs in HD can be divided into predominantly choreatic and hypokinetic-rigid subtypes. It has been reported in cross-sectional studies that predominantly choreatic HD patients perform better on functional and cognitive assessments compared to predominantly hypokinetic-rigid HD patients. The course of these motor subtypes and their clinical profiles has not been investigated longitudinally. A total of 4135 subjects who participated in the European HD Network REGISTRY study were included and classified at baseline as either predominantly choreatic (n = 891), hypokinetic-rigid (n = 916), or mixed-motor (n = 2328), based on a previously used method. The maximum follow-up period was 6 years. The mixed-motor group was not included in the analyses. Linear mixed models were constructed to investigate changes in motor subtypes over time and their relationship with cognitive and functional decline. Over the 6-year follow-up period, the predominantly choreatic group showed a significant decrease in chorea, while hypokinetic-rigid symptoms slightly increased in the hypokinetic-rigid group. On the Total Functional Capacity, Stroop test, and Verbal fluency task the rate of change over time was significantly faster in the predominantly choreatic group, while on all other clinical assessments the decline was comparable for both groups. Our results suggest that choreatic symptoms decrease over time, whereas hypokinetic-rigid symptoms slightly increase in a large cohort of HD patients. Moreover, different motor subtypes can be related to different clinical profiles

Latissimus dorsi rotational flap combined with a custom-made scapular prosthesis after oncological surgical resection: A report of two patients

BACKGROUND: Sarcomas that arise from the scapula or periscapular soft tissues often require a total scapulectomy. This often implies a large complex tissue defect that needs adequate reconstruction of both bone and soft tissue. Although various methods have been developed, no optimal procedure has emerged. Postoperative complications are common and functional recovery is not always satisfactory. This study aims to present a new surgical technique that combines a custom-made scapular prosthesis with a functional latissimus dorsi flap. CASE PRESENTATION: Two patients diagnosed with malignant tumour of the scapular region were surgically treated after proper multidisciplinary evaluation. The first patient underwent the procedure as a first surgery, the second as revision surgery. The new technique comprises three surgical stages: excisional surgery with soft tissue resection and scapulectomy, bone reconstruction with custom-made prosthesis, and soft tissue reconstruction using a latissimus dorsi rotational flap overturned on the prosthesis. The goal is to set up a new functional unit combining an anatomically shaped implant (manufactured using latest three-dimensional printing technology) and a muscular flap, and to maintain the neurovascular supply. The patients were followed up to evaluate functional outcome and complications. Both patients were alive with no evidence of disease. Functional results were satisfactory and the Musculoskeletal Tumor Society scores were 87% and 63%, respectively. No surgical complications such as implant breakage, joint collapse, wound dehiscence, or infection were observed. CONCLUSIONS: This new technique upgrades the role of the latissimus dorsi flap to a functional tool in combination with an anatomical, three-dimensionally printed, custom-made prosthesis, and provides adequate well-vascularized and healthy tissue to maximize the likelihood of successful limb salvage

An investigation of ozone and planetary boundary layer dynamics over the complex topography of Grenoble combining measurements and modeling

International audienceThis paper concerns an evaluation of ozone (O3) and planetary boundary layer (PBL) dynamics over the complex topography of the Grenoble region through a combination of measurements and mesoscale model (METPHOMOD) predictions for three days, during July 1999. The measurements of O3 and PBL structure were obtained with a Differential Absorption Lidar (DIAL) system, situated 20 km south of Grenoble at Vif (310 m a.s.l.). The combined lidar observations and model calculations are in good agreement with atmospheric measurements obtained with an instrumented aircraft (METAIR). Ozone fluxes were calculated using lidar measurements of ozone vertical profiles concentrations and the horizontal wind speeds measured with a Radar Doppler wind profiler (DEGREANE). The ozone flux patterns indicate that the diurnal cycle of ozone production is controlled by local thermal winds. The convective PBL maximum height was some 2700 m above the land surface while the nighttime residual ozone layer was generally found between 1200 and 2200 m. Finally we evaluate the magnitude of the ozone processes at different altitudes in order to estimate the photochemical ozone production due to the primary pollutants emissions of Grenoble city and the regional network of automobile traffic

Custom-made 3d-printed implants as novel approach to reconstructive surgery after oncologic resection in pediatric patients

Recently, custom-made 3D-printed prostheses have been introduced for limb salvage surgery in adult patients, but their use has not been described in pediatric patients. A series of 11 pediatric patients (mean age 10.8 years; range 2–13) with skeletal tumors treated with custom-made implants for the reconstruction of bony defects is described. Patients were followed up every 3 months. Functional results were evaluated by the Musculoskeletal Tumor Society Score (MSTS) for upper and lower limbs. The mean follow-up was 25.7 months (range 14–44). Three patients died after a mean of 19.3 months postoperatively—two because of disease progression and the other from a previous malignancy. Three patients experienced complications related to soft tissues. One patient required device removal, debridement, and antibiotic pearls for postoperative infection. Partial osseointegration between grafts and host bone was observed within a mean of 4 months. At the final follow-up, mean MSTS score was 75%. 3D prostheses may yield biological advantages due to possible integration with the host bone and also through the use of vascularized flaps. Further research is warranted

Explicit and implicit own’s body and space perception in painful musculoskeletal disorders and rheumatic diseases: a systematic scoping review

Background: Pain and body perception are essentially two subjective mutually influencing experiences. However, in the field of musculoskeletal disorders and rheumatic diseases we lack of a comprehensive knowledge about the relationship between body perception dysfunctions and pain or disability. We systematically mapped the literature published about the topics of: a) somatoperception; b) body ownership; and c) perception of space, analysing the relationship with pain and disability. The results were organized around the two main topics of the assessment and treatment of perceptual dysfunctions. Methods: This scoping review followed the six-stage methodology suggested by Arksey and O’Malley. Ten electronic databases and grey literature were systematically searched. The PRISMA Extension for Scoping Reviews was used for reporting results. Two reviewers with different background, independently performed study screening and selection, and one author performed data extraction, that was checked by a second reviewer. Results: Thirty-seven studies fulfilled the eligibility criteria. The majority of studies (68%) concerned the assessment methodology, and the remaining 32% investigated the effects of therapeutic interventions. Research designs, methodologies adopted, and settings varied considerably across studies. Evidence of distorted body experience were found mainly for explicit somatoperception, especially in studies adopting self-administered questionnaire and subjective measures, highlighting in some cases the presence of sub-groups with different perceptual features. Almost half of the intervention studies (42%) provided therapeutic approaches combining more than one perceptual task, or sensory-motor tasks together with perceptual strategies, thus it was difficult to estimate the relative effectiveness of each single therapeutic component. Conclusions: To our knowledge, this is the first attempt to systematically map and summarize this research area in the field of musculoskeletal disorders and rheumatic diseases. Although methodological limitations limit the validity of the evidence obtained, some strategies of assessment tested and therapeutic strategies proposed represent useful starting points for future research. This review highlights preliminary evidence, strengths, and limitations of the literature published about the research questions, identifying key points that remain opened to be addressed, and make suggestions for future research studies. Body representation, as well as pain perception and treatment, can be better understood if an enlarged perspective including body and space perception is considered

Explicit and implicit own’s body and space perception in painful musculoskeletal disorders and rheumatic diseases: a systematic scoping review

Background: Pain and body perception are essentially two subjective mutually influencing experiences. However, in the field of musculoskeletal disorders and rheumatic diseases we lack of a comprehensive knowledge about the relationship between body perception dysfunctions and pain or disability. We systematically mapped the literature published about the topics of: a) somatoperception; b) body ownership; and c) perception of space, analysing the relationship with pain and disability. The results were organized around the two main topics of the assessment and treatment of perceptual dysfunctions. Methods: This scoping review followed the six-stage methodology suggested by Arksey and O’Malley. Ten electronic databases and grey literature were systematically searched. The PRISMA Extension for Scoping Reviews was used for reporting results. Two reviewers with different background, independently performed study screening and selection, and one author performed data extraction, that was checked by a second reviewer. Results: Thirty-seven studies fulfilled the eligibility criteria. The majority of studies (68%) concerned the assessment methodology, and the remaining 32% investigated the effects of therapeutic interventions. Research designs, methodologies adopted, and settings varied considerably across studies. Evidence of distorted body experience were found mainly for explicit somatoperception, especially in studies adopting self-administered questionnaire and subjective measures, highlighting in some cases the presence of sub-groups with different perceptual features. Almost half of the intervention studies (42%) provided therapeutic approaches combining more than one perceptual task, or sensory-motor tasks together with perceptual strategies, thus it was difficult to estimate the relative effectiveness of each single therapeutic component. Conclusions: To our knowledge, this is the first attempt to systematically map and summarize this research area in the field of musculoskeletal disorders and rheumatic diseases. Although methodological limitations limit the validity of the evidence obtained, some strategies of assessment tested and therapeutic strategies proposed represent useful starting points for future research. This review highlights preliminary evidence, strengths, and limitations of the literature published about the research questions, identifying key points that remain opened to be addressed, and make suggestions for future research studies. Body representation, as well as pain perception and treatment, can be better understood if an enlarged perspective including body and space perception is considered