Background
Behavioural/functional disturbances, characteristic of frontotemporal dementia (FTD), are also a feature of amyotrophic lateral sclerosis (ALS) and patients with combined ALS and FTD ...(FTD‐ALS).
Aim of the study
To investigate the progression of behavioural disturbances in ALS and FTD using the FTD functional rating scale (FTDFRS).
Methods
Patients with ALS, FTD‐ALS and FTD were recruited from specialist clinics. Baseline assessments included the FTDFRS and the ALS functional rating scale–revised (ALSFRS‐R). Baseline assessments were included, as were longitudinal assessments in a proportion of patients.
Results
In total, 21 ALS, 12 FTD‐ALS and 14 behavioural variant FTD (bvFTD) patients were included in the study. Moderate or severe behavioural disturbance was common in patients with ALS at baseline (47.6%), although less frequent than in bvFTD patients; patients with FTD‐ALS displayed intermediate impairment. The ALSFRS‐R showed the opposite pattern and did not correlate with the FTDFRS. During the follow‐up period, significant (P < 0.05) behavioural deterioration was demonstrated in patients with bvFTD and FTD‐ALS, with a trend for decline in patients with ALS (P = 0.06).
Conclusion
Motor disturbance is the primary marker of disease severity in ALS, but behavioural and functional impairment are common, and may decline independently of motor function. As such, the FTDFRS may provide valuable information in the assessment and monitoring of ALS.
Neutrons produced in nuclear interactions initiated by cosmic-ray muons present an irreducible background to many rare-event searches, even in detectors located deep underground. Models for the ...production of these neutrons have been tested against previous experimental data, but the extrapolation to deeper sites is not well understood. Here we report results from an analysis of cosmogenically produced neutrons at the Sudbury Neutrino Observatory. A specific set of observables are presented, which can be used to benchmark the validity of geant4 physics models. In addition, the cosmogenic neutron yield, in units of 10−4 cm2/(g·μ), is measured to be 7.28±0.09(stat)−1.12+1.59(syst) in pure heavy water and 7.30±0.07(stat)−1.02+1.40(syst) in NaCl-loaded heavy water. These results provide unique insights into this potential background source for experiments at SNOLAB.
The Sudbury Neutrino Observatory (SNO) has measured day and night solar neutrino energy spectra and rates. For charged current events, assuming an undistorted 8B spectrum, the night minus day rate is ...14.0%+/-6.3%(+1.5%)(-1.4%) of the average rate. If the total flux of active neutrinos is additionally constrained to have no asymmetry, the nu(e) asymmetry is found to be 7.0%+/-4.9%(+1.3%)(-1.2%). A global solar neutrino analysis in terms of matter-enhanced oscillations of two active flavors strongly favors the large mixing angle solution.
Across different domains, there are 'star performers' who are able to generate disproportionate levels of performance output. To date, little is known about the model principles underlying the rise ...of star performers. Here, we propose that star performers' abilities develop according to a multi-dimensional, multiplicative and dynamical process. Based on existing literature, we defined a dynamic network model, including different parameters functioning as enhancers or inhibitors of star performance. The enhancers were multiplicity of productivity, monopolistic productivity, job autonomy, and job complexity, whereas productivity ceiling was an inhibitor. These enhancers and inhibitors were expected to influence the tail-heaviness of the performance distribution. We therefore simulated several samples of performers, thereby including the assumed enhancers and inhibitors in the dynamic networks and compared their tail-heaviness. Results showed that the dynamic network model resulted in heavier and lighter tail distributions, when including the enhancer- and inhibitor-parameters, respectively. Together, these results provide novel insights into the dynamical principles that give rise to star performers in the population.
The biliary clearance (Clbiliary) of three compounds was estimated using sandwich‐cultured human hepatocytes (SCHH) and compared with Clbiliary values measured in vivo. Tc‐99m sestamibi (MIBI) ...Clbiliary was determined in seven healthy volunteers using an oroenteric catheter to aspirate duodenal secretions, and gamma scintigraphy to determine gallbladder contraction; this technique was used previously to determine Tc‐99m mebrofenin (MEB) and piperacillin (PIP) in vivo Clbiliary. In vitro Clbiliary of MEB, MIBI, and PIP was quantified in SCHH as the ratio of mass excreted into bile canaliculi and area under the blood concentration‐time curve (AUC) in medium. MIBI Clbiliary in vivo was 5.5±1.2 mL/min/kg (mean±SD). The rank order of Clbiliary predicted from SCHH corresponded well with the in vivo Clbiliary values in mL/min/kg for MEB (7.44 vs 16.1), MIBI (1.20 vs 5.51), and PIP (0.028 vs 0.032). In conclusion, the methods developed allowed for reproducible quantification of Clbiliary of drugs in healthy humans and prediction of Clbiliary from in vitro data.
Clinical Pharmacology & Therapeutics (2007) 81, 406–413. doi:10.1038/sj.clpt.6100059; published online 18 January 2007
Edge effects have been studied for decades because they are a key component to understanding how landscape structure influences habitat quality. However, making sense of the diverse patterns and ...extensive variability reported in the literature has been difficult because there has been no unifying conceptual framework to guide research. In this review, we identify four fundamental mechanisms that cause edge responses: ecological flows, access to spatially separated resources, resource mapping, and species interactions. We present a conceptual framework that identifies the pathways through which these four mechanisms can influence distributions, ultimately leading to new ecological communities near habitat edges. Next, we examine a predictive model of edge responses and show how it can explain much of the variation reported in the literature. Using this model, we show that, when observed, edge responses are largely predictable and consistent. When edge responses are variable for the same species at the same edge type, observed responses are rarely in opposite directions. We then show how remaining variability may be understood within our conceptual frameworks. Finally, we suggest that, despite all the research in this area, the development of tools to extrapolate edge responses to landscapes has been slow, restricting our ability to use this information for conservation and management.
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