Assessment of motor symptoms and functional impact in prodromal and early Huntington disease.

The Functional Rating Scale Taskforce for pre-Huntington Disease (FuRST-pHD) is a multinational, multidisciplinary initiative with the goal of developing a data-driven, comprehensive, psychometrically sound, rating scale for assessing symptoms and functional ability in prodromal and early Huntington disease (HD) gene expansion carriers. The process involves input from numerous sources to identify relevant symptom domains, including HD individuals, caregivers, and experts from a variety of fields, as well as knowledge gained from the analysis of data from ongoing large-scale studies in HD using existing clinical scales. This is an iterative process in which an ongoing series of field tests in prodromal (prHD) and early HD individuals provides the team with data on which to make decisions regarding which questions should undergo further development or testing and which should be excluded. We report here the development and assessment of the first iteration of interview questions aimed to assess functional impact of motor manifestations in prHD and early HD individuals.

An item response analysis of the motor and behavioral subscales of the unified Huntington's disease rating scale in huntington disease gene expansion carriers

Although the Unified Huntington's Disease Rating Scale (UHDRS) is widely used in the assessment of Huntington disease (HD), the ability of individual items to discriminate individual differences in motor or behavioral manifestations has not been extensively studied in HD gene expansion carriers without a motor-defined clinical diagnosis (ie, prodromal-HD or prHD). To elucidate the relationship between scores on individual motor and behavioral UHDRS items and total score for each subscale, a nonparametric item response analysis was performed on retrospective data from 2 multicenter longitudinal studies. Motor and behavioral assessments were supplied for 737 prHD individuals with data from 2114 visits (PREDICT-HD) and 686 HD individuals with data from 1482 visits (REGISTRY). Option characteristic curves were generated for UHDRS subscale items in relation to their subscale score. In prHD, overall severity of motor signs was low, and participants had scores of 2 or above on very few items. In HD, motor items that assessed ocular pursuit, saccade initiation, finger tapping, tandem walking, and to a lesser extent, saccade velocity, dysarthria, tongue protrusion, pronation/supination, Luria, bradykinesia, choreas, gait, and balance on the retropulsion test were found to discriminate individual differences across a broad range of motor severity. In prHD, depressed mood, anxiety, and irritable behavior demonstrated good discriminative properties. In HD, depressed mood demonstrated a good relationship with the overall behavioral score. These data suggest that at least some UHDRS items appear to have utility across a broad range of severity, although many items demonstrate problematic features.

Health-related quality of life in Huntington’s Disease patients: a comparison of proxy assessment and patient self-rating using the disease-specific Huntington’s Disease health-related quality of life questionnaire (HDQoL)

Huntington’s disease (HD) is a fatal, neurodegenerative disease for which there is no known cure. Proxy evaluation is relevant for HD as its manifestation might limit the ability of persons to report their health-related quality of life (HrQoL). This study explored patient–proxy ratings of HrQoL of persons at different stages of HD, and examined factors that may affect proxy ratings. A total of 105 patient–proxy pairs completed the Huntington’s disease health-related quality of life questionnaire (HDQoL) and other established HrQoL measures (EQ-5D and SF-12v2). Proxy–patient agreement was assessed in terms of absolute level (mean ratings) and intraclass correlation. Proxies’ ratings were at a similar level to patients’ self-ratings on an overall Summary Score and on most of the six Specific Scales of the HDQoL. On the Specific Hopes and Worries Scale, proxies on average rated HrQoL as better than patients’ self-ratings, while on both the Specific Cognitive Scale and Specific Physical and Functional Scale proxies tended to rate HrQoL more poorly than patients themselves. The patient’s disease stage and mental wellbeing (SF-12 Mental Component scale) were the two factors that primarily affected proxy assessment. Proxy scores were strongly correlated with patients’ self-ratings of HrQoL, on the Summary Scale and all Specific Scales. The patient–proxy correlation was lower for patients at moderate stages of HD compared to patients at early and advanced stages. The proxy report version of the HDQoL is a useful complementary tool to self-assessment, and a promising alternative when individual patients with advanced HD are unable to self-report.

A proposal for a new scenario framework to support research and assessment in different climate research communities

In this paper, we propose a scenario framework that could provide a scenario “thread” through the different climate research communities (climate change – vulnerability, impact, and adaptation (VIA) and mitigation) in order to provide assessment of mitigation and adaptation strategies and other VIA challenges. The scenario framework is organised around a matrix with two main axes: radiative forcing levels and socio-economic conditions. The radiative forcing levels (and the associated climate signal) are described by the new Representative Concentration Pathways. The second axis, socio-economic developments, comprises elements that affect the capacity for mitigation and adaptation, as well as the exposure to climate impacts. The proposed scenarios derived from this framework are limited in number, allow for comparison across various mitigation and adaptation levels, address a range of vulnerability characteristics, provide information across climate forcing and vulnerability states and span a full century time scale. Assessments based on the proposed scenario framework would strengthen cooperation between integrated-assessment modelers, climate modelers and vulnerability, impact and adaptation researchers, and most importantly, facilitate the development of more consistent and comparable research within and across communities.

Measuring societal awareness of the rural agrarian landscape: indicators and scale issues

The work presented in this report is part of the effort to define the landscape state and diversity indicator in the frame of COM (2006) 508 “Development of agri-environmental indicators for monitoring the integration of environmental concerns into the common agricultural policy”. The Communication classifies the indicators according to their level of development, which, for the landscape indicator is “in need of substantial improvements in order to become fully operational”. For this reason a full re-definition of the indicator has been carried out, following the initial proposal presented in the frame of the IRENA operation (“Indicator Reporting on the Integration of Environmental Concerns into Agricultural Policy”). The new proposal for the landscape state and diversity indicator is structured in three components: the first concerns the degree of naturalness, the second landscape structure, the third the societal appreciation of the rural landscape. While the first two components rely on a strong bulk of existing literature, the development of the methodology has made evident the need for further analysis of the third component, which is based on a newly proposed top-down approach. This report presents an in-depth analysis of such component of the indicator, and the effort to include a social dimension in large scale landscape assessment.

Integrating nitrogen fluxes at the European scale

Executive summary Nature of the problem • Environmental problems related to nitrogen concern all economic sectors and impact all media: atmosphere, pedosphere, hydrosphere and anthroposphere. • Therefore, the integration of fluxes allows an overall coverage of problems related to reactive nitrogen (Nr) in the environment, which is not accessible from sectoral approaches or by focusing on specific media. Approaches • This chapter presents a set of high resolution maps showing key elements of the N flux budget across Europe, including N2 and Nr fluxes. • Comparative nitrogen budgets are also presented for a range of European countries, highlighting the most efficient strategies for mitigating Nr problems at a national scale. A new European Nitrogen Budget (EU-27) is presented on the basis of state-of-the-art Europe-wide models and databases focusing on different segments of Europe’s society. Key findings • From c. 18 Tg Nr yr −1 input to agriculture in the EU-27, only about 7 Tg Nr yr− 1 find their way to the consumer or are further processed by industry. • Some 3.7 Tg Nr yr−1 is released by the burning of fossil fuels in the EU-27, whereby the contribution of the industry and energy sectors is equal to that of the transport sector. More than 8 Tg Nr yr−1 are disposed of to the hydrosphere, while the EU-27 is a net exporter of reactive nitrogen through atmospheric transport of c. 2.3 Tg Nr yr−1. • The largest single sink for Nr appears to be denitrifi cation to N2 in European coastal shelf regions (potentially as large as the input of mineral fertilizer, about 11 Tg N yr–1 for the EU-27); however, this sink is also the most uncertain, because of the uncertainty of Nr import from the open ocean. Major uncertainties • National nitrogen budgets are diffi cult to compile using a large range of data sources and are currently available only for a limited number of countries. • Modelling approaches have been used to fill in the data gaps in some of these budgets, but it became obvious during this study that further research is needed in order to collect necessary data and make national nitrogen budgets inter-comparable across Europe. • In some countries, due to inconsistent or contradictory information coming from different data sources, closure of the nitrogen budget was not possible. Recommendations • The large variety of problems associated with the excess of Nr in the European environment,including adverse impacts, requires an integrated nitrogen management approach that would allow for creation and closure of N budgets within European environments. • Development of nitrogen budgets nationwide, their assessment and management could become an effective tool to prioritize measures and prevent unwanted side effects.

The use of public participation and economic appraisal for public involvement in large-scale hydropower projects: Case study of the Nam Theun 2 Hydropower Project

Gaining public acceptance is one of the main issues with large-scale low-carbon projects such as hydropower development. It has been recommended by the World Commission on Dams that to gain public acceptance, publicinvolvement is necessary in the decision-making process (WCD, 2000). As financially-significant actors in the planning and implementation of large-scale hydropowerprojects in developing country contexts, the paper examines the ways in which publicinvolvement may be influenced by international financial institutions. Using the casestudy of the NamTheun2HydropowerProject in Laos, the paper analyses how publicinvolvement facilitated by the Asian Development Bank had a bearing on procedural and distributional justice. The paper analyses the extent of publicparticipation and the assessment of full social and environmental costs of the project in the Cost-Benefit Analysis conducted during the projectappraisal stage. It is argued that while efforts were made to involve the public, there were several factors that influenced procedural and distributional justice: the late contribution of the Asian Development Bank in the projectappraisal stage; and the issue of non-market values and discount rate to calculate the full social and environmental costs.

Variation in the global-scale impacts of climate change on crop productivity due to climate model uncertainty and adaptation

Crop production is inherently sensitive to fluctuations in weather and climate and is expected to be impacted by climate change. To understand how this impact may vary across the globe many studies have been conducted to determine the change in yield of several crops to expected changes in climate. Changes in climate are typically derived from a single to no more than a few General Circulation Models (GCMs). This study examines the uncertainty introduced to a crop impact assessment when 14 GCMs are used to determine future climate. The General Large Area Model for annual crops (GLAM) was applied over a global domain to simulate the productivity of soybean and spring wheat under baseline climate conditions and under climate conditions consistent with the 2050s under the A1B SRES emissions scenario as simulated by 14 GCMs. Baseline yield simulations were evaluated against global country-level yield statistics to determine the model's ability to capture observed variability in production. The impact of climate change varied between crops, regions, and by GCM. The spread in yield projections due to GCM varied between no change and a reduction of 50%. Without adaptation yield response was linearly related to the magnitude of local temperature change. Therefore, impacts were greatest for countries at northernmost latitudes where warming is predicted to be greatest. However, these countries also exhibited the greatest potential for adaptation to offset yield losses by shifting the crop growing season to a cooler part of the year and/or switching crop variety to take advantage of an extended growing season. The relative magnitude of impacts as simulated by each GCM was not consistent across countries and between crops. It is important, therefore, for crop impact assessments to fully account for GCM uncertainty in estimating future climates and to be explicit about assumptions regarding adaptation.

A global assessment of the effects of climate policy on the impacts of climate change

This study presents the first global-scale multi-sectoral regional assessment of the magnitude and uncertainty in the impacts of climate change avoided by emissions policies. The analysis suggests that the most stringent emissions policy considered here – which gives a 50% chance of remaining below a 2oC temperature rise target - reduces impacts by 20-65% by 2100 relative to a ‘business-as-usual’ pathway (A1B) which reaches 4oC, and can delay impacts by several decades. Effects vary between sector and region, and there are few noticeable effects of mitigation policy by 2030. The impacts avoided by 2100 are more strongly influenced by the date and level at which emissions peak than the rate of decline of emissions, with an earlier and lower emissions peak avoiding more impacts. The estimated proportion of impacts avoided at the global scale is relatively robust despite uncertainty in the spatial pattern of climate change, but the absolute amount of avoided impacts is considerably more variable and therefore uncertain.

A parentally-administered cognitive development assessment for children from 10 to 24 months

The Cognitive Development Questionnaire (CDQ) allows accurate assessment of cognitive development of children from 10 to 24 months by parents and caregivers in the home. It takes between one and two hours to complete over about a week. Three phases of work are described, in which the instrument is progressively refined to improve its validity and reliability. This resulting version of the CDQ shows excellent correlation with age, and with the Mental Scale of the Bayley Scales of Infant Development (Bayley, 1993). The CDQ thus offers researchers and clinicians a useful alternative to professionally-administered cognitive assessment in infancy.

Identifying time lags in the restoration of grassland butterfly communities: a multi-site assessment

Although grasslands are crucial habitats for European butterflies, large-scale declines in quality and area have devastated many species. Grassland restoration can contribute to the recovery of butterfly populations, although there is a paucity of information on the long-term effects of management. Using eight UK data sets (9-21 years), we investigate changes in restoration success for (1) arable reversion sites, were grassland was established on bare ground using seed mixtures, and (2) grassland enhancement sites, where degraded grasslands are restored by scrub removal followed by the re-instigation of cutting/grazing. We also assessed the importance of individual butterfly traits and ecological characteristics in determining colonisation times. Consistent increases in restoration success over time were seen for arable reversion sites, with the most rapid rates of increase in restoration success seen over the first 10 years. For grasslands enhancement there were no consistent increases in restoration success over time. Butterfly colonisation times were fastest for species with widespread host plants or where host plants established well during restoration. Low mobility butterfly species took longer to colonise. We show that arable reversion is an effective tool for the management of butterfly communities. We suggest that as restoration takes time to achieve, its use as a mitigation tool against future environmental change (i.e. by decreasing isolation in fragmented landscapes) needs to take into account such time lags.

The impacts of climate change on river flow regimes at the global scale

This paper presents an assessment of the impacts of climate change on a series of indicators of hydrological regimes across the global domain, using a global hydrological model run with climate scenarios constructed using pattern-scaling from 21 CMIP3 (Coupled Model Intercomparison Project Phase 3) climate models. Changes are compared with natural variability, with a significant change being defined as greater than the standard deviation of the hydrological indicator in the absence of climate change. Under an SRES (Special Report on Emissions Scenarios) A1b emissions scenario, substantial proportions of the land surface (excluding Greenland and Antarctica) would experience significant changes in hydrological behaviour by 2050; under one climate model scenario (Hadley Centre HadCM3), average annual runoff increases significantly over 47% of the land surface and decreases over 36%; only 17% therefore sees no significant change. There is considerable variability between regions, depending largely on projected changes in precipitation. Uncertainty in projected river flow regimes is dominated by variation in the spatial patterns of climate change between climate models (hydrological model uncertainty is not included). There is, however, a strong degree of consistency in the overall magnitude and direction of change. More than two-thirds of climate models project a significant increase in average annual runoff across almost a quarter of the land surface, and a significant decrease over 14%, with considerably higher degrees of consistency in some regions. Most climate models project increases in runoff in Canada and high-latitude eastern Europe and Siberia, and decreases in runoff in central Europe, around the Mediterranean, the Mashriq, central America and Brasil. There is some evidence that projecte change in runoff at the regional scale is not linear with change in global average temperature change. The effects of uncertainty in the rate of future emissions is relatively small

Assessment of a 1 hour gridded precipitation dataset to drive a hydrological model: a case study of the summer 2007 floods in the Upper Severn, UK

In this study a gridded hourly 1-km precipitation dataset for a meso-scale catchment (4,062 km2) of the Upper Severn River, UK was constructed using rainfall radar data to disaggregate a daily precipitation (rain gauge) dataset. The dataset was compared to an hourly precipitation dataset created entirely from rainfall radar data. Results found that when assessed against gauge readings and as input to the Lisflood-RR hydrological model, the rain gauge/radar disaggregated dataset performed the best suggesting that this simple method of combining rainfall radar data with rain gauge readings can provide temporally detailed precipitation datasets for calibrating hydrological models.

Assessment of temperature, trace species, and ozone in chemistry-climate model simulations of the recent past

Simulations of the stratosphere from thirteen coupled chemistry-climate models (CCMs) are evaluated to provide guidance for the interpretation of ozone predictions made by the same CCMs. The focus of the evaluation is on how well the fields and processes that are important for determining the ozone distribution are represented in the simulations of the recent past. The core period of the evaluation is from 1980 to 1999 but long-term trends are compared for an extended period (1960–2004). Comparisons of polar high-latitude temperatures show that most CCMs have only small biases in the Northern Hemisphere in winter and spring, but still have cold biases in the Southern Hemisphere spring below 10 hPa. Most CCMs display the correct stratospheric response of polar temperatures to wave forcing in the Northern, but not in the Southern Hemisphere. Global long-term stratospheric temperature trends are in reasonable agreement with satellite and radiosonde observations. Comparisons of simulations of methane, mean age of air, and propagation of the annual cycle in water vapor show a wide spread in the results, indicating differences in transport. However, for around half the models there is reasonable agreement with observations. In these models the mean age of air and the water vapor tape recorder signal are generally better than reported in previous model intercomparisons. Comparisons of the water vapor and inorganic chlorine (Cly) fields also show a large intermodel spread. Differences in tropical water vapor mixing ratios in the lower stratosphere are primarily related to biases in the simulated tropical tropopause temperatures and not transport. The spread in Cly, which is largest in the polar lower stratosphere, appears to be primarily related to transport differences. In general the amplitude and phase of the annual cycle in total ozone is well simulated apart from the southern high latitudes. Most CCMs show reasonable agreement with observed total ozone trends and variability on a global scale, but a greater spread in the ozone trends in polar regions in spring, especially in the Arctic. In conclusion, despite the wide range of skills in representing different processes assessed here, there is sufficient agreement between the majority of the CCMs and the observations that some confidence can be placed in their predictions.

Global to city scale urban anthropogenic heat flux: model and variability

The large scale urban consumption of energy (LUCY) model simulates all components of anthropogenic heat flux (QF) from the global to individual city scale at 2.5 × 2.5 arc-minute resolution. This includes a database of different working patterns and public holidays, vehicle use and energy consumption in each country. The databases can be edited to include specific diurnal and seasonal vehicle and energy consumption patterns, local holidays and flows of people within a city. If better information about individual cities is available within this (open-source) database, then the accuracy of this model can only improve, to provide the community data from global-scale climate modelling or the individual city scale in the future. The results show that QF varied widely through the year, through the day, between countries and urban areas. An assessment of the heat emissions estimated revealed that they are reasonably close to those produced by a global model and a number of small-scale city models, so results from LUCY can be used with a degree of confidence. From LUCY, the global mean urban QF has a diurnal range of 0.7–3.6 W m−2, and is greater on weekdays than weekends. The heat release from building is the largest contributor (89–96%), to heat emissions globally. Differences between months are greatest in the middle of the day (up to 1 W m−2 at 1 pm). December to February, the coldest months in the Northern Hemisphere, have the highest heat emissions. July and August are at the higher end. The least QF is emitted in May. The highest individual grid cell heat fluxes in urban areas were located in New York (577), Paris (261.5), Tokyo (178), San Francisco (173.6), Vancouver (119) and London (106.7). Copyright © 2010 Royal Meteorological Society