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Multiple System Atrophy Without Dysautonomia : An Autopsy-Confirmed Study
No full textBACKGROUND AND OBJECTIVES: Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by 3 core symptom complexes: parkinsonism, cerebellar syndrome, and dysautonomia. Recent Movement Disorder Society (MDS) criteria allow for the clinical diagnosis of MSA based solely on motor symptoms, without requiring dysautonomia. This study aimed to evaluate the frequency and disease trajectory of MSA patients without dysautonomia compared with those with autonomic involvement. METHODS: A multicenter cohort of autopsy-confirmed patients with MSA was analyzed for demographic characteristics, symptom onset, and progression of parkinsonism, cerebellar syndrome, and dysautonomia. Clinical data were collected through standardized chart reviews across participating centers and categorized using the MDS-MSA criteria. Patients were grouped according to their initial symptom complex and tracked for the evolution of additional symptoms. Analyses included time to development of further symptom complexes, age at symptom onset, disease duration, and phenotype at the last recorded visit. Patients with motor symptoms only were matched to patients with similar demographics but with dysautonomia. Statistical methods included ANOVA, t tests, Welch t tests, and χ2 tests, with significance set at p < 0.05. RESULTS: Among 140 patients (mean age at onset 62.3 ± 8.9 years; 44% female), 81 (58%) initially presented without dysautonomia (57 with parkinsonism only, 17 with cerebellar syndrome only, 7 with both). At final follow-up, 12 patients (9%) had not developed dysautonomia. These patients showed significantly longer disease duration (mean 8.1 ± 2.1 years) than matched patients with dysautonomia (mean 6.3 ± 2.6 years; p = 0.035). Overall, 51% of patients developed all 3 symptom complexes. Patients with cerebellar onset progressed more rapidly to multisystem involvement than those with parkinsonian onset (mean interval to second symptom: 2.0 vs 3.4 years; p < 0.05). DISCUSSION: The MDS-MSA criteria expand the diagnostic scope by identifying a motor-only subgroup with a distinct and potentially slower disease course. These findings underscore the importance of including motor-only patients in natural history and interventional studies. Limitations include retrospective data collection and potential variability in symptom documentation
Injuries, illnesses and athlete health in para athletes
No full textPara athletes are at higher risk for sports-related injuries, illnesses and other health conditions when participating in sports. In fact, the unique demands placed on Para athletes, both in terms of the specific impairments they experience and the adaptive equipment they use, can lead to a distinctive set of challenges and risks in the context of sports-related health compared to nondisabled athletes.It has been suggested that the impairment itself should be considered as an intrinsic risk factor for a sports-related injury or illness. This chapter presents the most important impairment-related mechanisms that may contribute to an injury or illness for the most common athlete impairment categories. Furthermore, this chapter highlights the need for pre-participation screening, athlete health monitoring and prevention of sports injuries and illnesses in Para sport
The Strategic Making of Sustainable Venice
No full textDrawing on a constitutive understanding of strategic communication [Falkheimer and Heide (Strategic communication: An introduction. Routledge, 2018), (Strategic communication: An introduction to theory and global practice. Routledge, 2022); Winkler and Schoeneborn (The research handbook of strategic communication. Edward Elgar, 2023)], this chapter examines how the municipality of Venice’s strategic sustainability communication not only represents the city’s sustainability practices but also constitutes what a sustainable Venice means in the first place. In particular, it analyses a communication campaign to inform tourists about flooding events as well as an interview with a local politician. The findings show that the making of a sustainable Venice is the result of a negotiation of the meaning of sustainability between the specificities of the local urban setting and the meaning found in the global discourse. Extending analyses of the strategic use of language to communicate about sustainability, this chapter shows how strategic sustainability communication is a process that involves both crafted and emergent instances of strategic communication in the constitution of sustainable places. As such, it helps to circumvent some of the limitations of representational understandings of strategic sustainability communication by showing how such practice is inherently political, influencing the transformation of urban places in the age of climate change
Longitudinal decline in upper-limb range of motion in adults with cerebral palsy
No full textTo analyse longitudinal changes in passive range of motion (ROM) in the upper limb in adults with cerebral palsy (CP). Method: Passive ROM for shoulder abduction and flexion, supination, and elbow and wrist extension was analysed in a longitudinal cohort of adults aged 16 to 76 years from the Swedish CP registry. Individual ROM trajectories and mean ROM curves were calculated using the Manual Ability Classification System (MACS). A mixed-effects model was used to examine changes over 3 to 13 years 7 months. Results: In total, 1395 adults with CP were analysed (769 males, 626 females; median age 26 years). A continuous decline in shoulder ROM, supination, and wrist extension was observed across all MACS levels. Decline rates differed between MACS levels for shoulder flexion, elbow extension, and wrist extension, with steeper declines at higher MACS levels (levels IV and V). Adults classified in lower MACS levels (I and II) had greater initial ROM and slower declines compared to adults classified in higher MACS levels. Interpretation. Upper-limb ROM continuously declined in adults with CP, particularly at higher MACS levels. The varied decline rates highlight the need for tailored interventions and systematic follow-up to maintain ROM and functional ability, especially among individuals at higher risk
Multigrain Ruthenium Nanocrystals with Enriched (101¯1) Facets for Enhanced Hydrogen Oxidation in Anion Exchange Membrane Fuel Cells
No full textRuthenium-based materials are promising alternatives to expensive platinum for the anodic hydrogen oxidation reaction (HOR) in anion exchange membrane fuel cells (AEMFCs), but face stability issues due to the strong oxophilicity. Here, an oxidation-resistant ruthenium multigrain catalyst is reported that exposes rich (10 (Formula presented.) 1) facets for high-performing HOR catalysis in alkaline electrolytes. The catalyst exhibits a high kinetic current density of 61 mA cm−2 at an overpotential of 50 mV, which is 25.6- and 7.8-times higher than that of commercial ruthenium-carbon and platinum-carbon catalysts, respectively. Moreover, it also demonstrates a wide stability window up to 0.3 V versus the reverse hydrogen electrode and enhanced tolerance to carbon monoxide. An AEMFC containing this catalyst at the anode achieves peak power densities of 1.31 and 1.06 W cm−2 under hydrogen-oxygen and hydrogen-air conditions at 90 °C, respectively, and operates steadily. Experimental and theoretical studies reveal that the (10 (Formula presented.) 1) facet possesses a higher oxidation barrier and lower hydrogen oxidation barrier than the common (0002) facets, enabling the exceptional HOR performances in alkali
Evaluating the In-Plane Shear Capacity of Tongue-and-Groove Panels
No full textThis study evaluates the in-plane shear capacity of timber tongue-and-groove (TG) panels commonly used for roof structures in Nordic countries. Experimental tests, including shear and racking strength assessments, were conducted to investigate the in-plane capacity and the contribution of corrugated fasteners (pins) to structural performance. Racking strength tests demonstrated improved load-bearing capacity in panelised configurations, with reinforced TG panels achieving almost diaphragm action, though not as stiff as plywood. Shear tests showed a linear increase in capacity with additional pins, with each pin contributing 1 kN. Results revealed limitations in existing theoretical models, which overestimate failure loads by neglecting deformation. Findings highlight the importance of fasteners in improving strength, providing valuable insights for safer timber construction
The devil is in the details (?) : Age-based discrimination of children in the context of migration
No full textDesign and Implementation of the Correntropy-Based Filter for GNSS Vector Tracking and Positioning
No full textIn recent decades, a significant advancement in the Global Navigation Satellite System (GNSS) has been adopting the vector tracking (VT) technique, particularly in environments where stable and reliable positioning is essential. It facilitates rapid reacquisition and continuous tracking of GNSS signals by linking the data processing and signal processing modules. In challenging environments affected by multipath and non-line-of-sight errors, such interaction adversely degrades VT’s performance by introducing non-Gaussian error propagation between the navigation processor and baseband channels. This study presents a robust filtering approach based on maximum correntropy criterion optimization to address the above challenges. A multikernel assignment strategy has also been formulated to enhance VT stability in demanding scenarios. Extensive tests have been conducted to assess the performance and efficacy of the proposed methodology. The results indicate that the proposed filter model significantly reduces positioning errors in static and dynamic scenarios. Moreover, the method demonstrates resilience and reliability across diverse urban settings
Predicting neoadjuvant chemotherapy treatment response in hormone-receptor-positive/HER2-negative breast cancer – results from the Swedish SCAN-B population-based cohort
No full textBackground and purpose: Hormone-receptor-positive/HER2-negative (HR+/HER2-) early-stage breast cancers (EBCs) display heterogenous responses to neoadjuvant chemotherapy (NACT) warranting biomarkers to tailor optimal treatment for individual patients. Patients/material and methods: Women with HR+/HER2-EBC (N = 178) included in the Swedish Sweden Cancerome Analysis Network-Breast (SCAN-B) population-based cohort (2010–2019) treated with NACT were included. We analyzed rates of pathologic complete response (pCR), objective response (OR), breast conserving surgery (BCS), and recurrence-free interval (RFI) in subgroups defined by baseline clinicopath-ological and molecular characteristics. Results: The pCR rate was low (6%); nonetheless, after a median follow-up of 5.41 years, all patients who achieved pCR remained recurrence-free despite uniform baseline predicted high PAM50 risk of recurrence (ROR). Younger age (≤ 40 years), cT1, ER% positivity (≤ 66%), and negative PR (≤ 10%) were conventional clinicopathological factors positively associated with increased pCR. Molecular predictors of pCR included negative HR status by gene-expression signatures and non-luminal PAM50 subtypes. Tumor shrinkage ≥ 30%, i.e., OR and BCS, was achieved in 59% and 34%, respectively. No factor was significantly associated with ORR, whereas non-lobular histology and cT1 were positively associated with BCS. In addition, only 1/49 patients who underwent BCS experienced a recurrence during follow-up. Low/intermediate ER% pos-itivity, PR negativity, and non-luminal PAM50 subtype were baseline factors univariately prognostic for inferior long-term outcome in case of residual disease. Interpretation: Baseline characteristics indicative of reduced hormonal signaling and non-luminal tumor biology assessed more precisely using mRNA profiling can guide optimal tailoring of NACT for patients with high-risk HR+/HER2-tumors. Baseline molecular biology did not predict surgical outcomes following NACT