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.

Eco-labeling, rents, sales prices and occupancy rates: do LEED and Energy Star labeled offices obtain multiple premiums?

Drawing upon an updated and expanded dataset of Energy Star and LEED labeled commercial offices, this paper investigates the effect of eco-labeling on rental rates, sale prices and occupancy rates. Using OLS and robust regression procedures, hedonic modeling is used to test whether the presence of an eco-label has a significant positive effect on rental rates, sale prices and occupancy rates. The study suggests that estimated coefficients can be sensitive to outlier treatment. For sale prices and occupancy rates, there are notable differences between estimated coefficients for OLS and robust regressions. The results suggest that both Energy Star and LEED offices obtain rental premiums of approximately 3%. A 17% sale price premium is estimated for Energy Star labeled offices but no significant sale price premium is estimated for LEED labeled offices. Surprisingly, no significant occupancy premium is estimated for Energy Star labeled offices and a negative occupancy premium is estimated for LEED labeled offices.

The information content of REIT credit rating actions and transparency

We examine the short-run and long-run price reaction of equity REIT shares following credit rating actions, testing the transparency of the REIT structure. Generally, the economic effect on the stock price is subdued for both upgrades and downgrades compared to prior literature examining the broader U.S. equity market. An examination of trading volume revealed a significant increase in trading in reaction to downgrade credit rating changes, with a more subdued response to upgrades. The findings support the notion that REITs are more publicly forthcoming about the expectation of positive news in comparison to negative news.

Synthesis and crystal structure of a novel octa-aqua bridged star-shaped Ni4K complex

A penta-nuclear. star-shaped hetero-metallic compound containing a unique Ni4KO8 core has been synthesized. The X-ray single crystal structure analysis reveals that in the complex, [K(Ni(LH)(2))(4)(OH2)(8)](Br)(ClO4)(8)center dot 11H(2)O (LH=(CH3)(2)HN+(CH2)(3)N=CHC6H4O-) the eight coordinate central K+ ion is encapsulated by four terminal [Ni(LH)(2)](2+) units through the double water bridges between K+ and each Ni(II) ions.

Can environmental performance rating programmes succeed in Africa?: an evaluation of Ghana’s AKOBEN project

The purpose of the article is to describe and analyse Ghana’s AKOBEN programme which is the first environmental performance rating and public disclosure programme in Africa. Furthermore, by means of a SWOT analysis, the article assesses the suitability of AKOBEN as a veritable tool for promoting good environmental governance in Ghana specifically and Africa in general.

The effect of the magnetic field of the secondary star in dwarf novae

We investigate the effect of a secondary star magnetic field on the accretion disc dynamics of dwarf novae. Simulations have been carried out with a particle code and a dipolar magnetic field structure. The magnetic field acts to remove angular momentum from the disc material, increasing the inward mass flow. This makes the accretion disc more centrally condensed, causing a reduction in the recurrence time for dwarf nova outbursts. We have produced Doppler tomograms and light curves which may be compared with observations. These tomograms are significantly different from those produced in the absence of a magnetic field on the secondary. We derive an upper limit to the magnetic moment of the secondary star in UGem of mu_2<2x10^32 A m^2. The magnetic truncation of the accretion disc produces resonance phenomena similar to those seen in the superoutbursts of SUUMa systems. While these have not been observed for systems like UGem, observations of the SUUMa systems provide us with a useful diagnostic of the disc-field interaction. We are able to place an upper limit on the magnetic moment of the secondary in ZCha of mu_2<1x10^30 A m^2.

Star/poverty space: the making of the ‘development celebrity’

What is it that gives celebrities the voice and authority to do and say the things they do in the realm of development politics? Asked another way, how is celebrity practised and, simultaneously, how does this praxis make celebrity, personas, politics and, indeed, celebrities themselves? In this article, we explore this ‘celebrity praxis’ through the lens of the creation of the contemporary ‘development celebrity’ in those stars working for development writ large in the so-called Third World. Drawing on work in science studies, material cultures and the growing geo-socio-anthropologies of things, the key to understanding the material practices embedded in and creating development celebrity networks is the multiple and complex circulations of the everyday and bespectacled artefacts of celebrity. Conceptualised as the ‘celebrity–consumption–compassion complex’, the performances of development celebrities are as much about everyday events, materials, technologies, emotions and consumer acts as they are about the mediated and liquidised constructions of the stars who now ‘market’ development.Moreover, this complex is constructed by and constructs what we are calling ‘star/poverty space’ that works to facilitate the ‘expertise’ and ‘authenticity’ and, thus, elevated voice and authority, of development celebrities through poverty tours, photoshoots, textual and visual diaries, websites and tweets. In short, the creation of star/poverty space is performed through a kind of ‘materiality of authenticity’ that is at the centre of the networks of development celebrity. The article concludes with several brief observations about the politics, possibilities and problematics of development celebrities and the star/poverty spaces that they create.

Approximation by harmonic polynomials in star-shaped domains and exponential convergence of Trefftz hp-dGFEM

We study the approximation of harmonic functions by means of harmonic polynomials in two-dimensional, bounded, star-shaped domains. Assuming that the functions possess analytic extensions to a delta-neighbourhood of the domain, we prove exponential convergence of the approximation error with respect to the degree of the approximating harmonic polynomial. All the constants appearing in the bounds are explicit and depend only on the shape-regularity of the domain and on delta. We apply the obtained estimates to show exponential convergence with rate O(exp(−b square root N)), N being the number of degrees of freedom and b>0, of a hp-dGFEM discretisation of the Laplace equation based on piecewise harmonic polynomials. This result is an improvement over the classical rate O(exp(−b cubic root N )), and is due to the use of harmonic polynomial spaces, as opposed to complete polynomial spaces.

Comparative Cluster/Double Star observations of the high and low latitude dayside magnetopause

The launch of the Double Star mission has provided the opportunity to monitor events at distinct locations on the dayside magnetopause, in coordination with the quartet of Cluster spacecraft. We present results of two such coordinated studies. In the first, 6 April 2004, both Cluster and the Double Star TC-1 spacecraft were on outbound transits through the dawn-side magnetosphere. Cluster observed northward moving FTEs with +/- polarity, whereas TC-1 saw -/+ polarity FTEs. The strength, motion and occurrence of the FTE signatures changes somewhat according to changes in IMF clock angle. These observations are consistent with ongoing reconnection on the dayside magnetopause, resulting in a series of flux transfer events (FTEs) seen both at Cluster and TC-1. The observed polarity and motion of each FTE signature advocates the existence of an active reconnection region consistently located between the positions of Cluster and TC-1, lying north and south of the reconnection line, respectively. This scenario is supported by the application of a model, designed to track flux tube motion, to conditions appropriate for the prevailing interplanetary conditions. The results from the model confirm the observational evidence that the low-latitude FTE dynamics is sensitive to changes in convected upstream conditions. In particular, changing the interplanetary magnetic field (IMF) clock angle in the model predicts that TC-1 should miss the resulting FTEs more often than Cluster, as is observed. For the second conjunction, on the 4 Jan 2005, the Cluster and TC-1 spacecraft all exited the dusk-side magnetosphere almost simultaneously, with TC-1 lying almost equatorial and Cluster at northern latitudes at about 4 RE from TC-1. The spacecraft traverse the magnetopause during a strong reversal in the IMF from northward to southward and a number of magnetosheath FTE signatures are subsequently observed. One coordinated FTE, studied in detail by Pu et al, [this issue], carries and inflowing energetic electron population and shows a motion and orientation which is similar at all spacecraft and consistent with the predictions of the model for the flux tube dynamics, given a near sub-solar reconnection line. This event can be interpreted either as the passage of two parallel flux tubes arising from adjacent x-line positions, or as a crossing of a single flux tube at different positions.

Coordinated Cluster/Double Star observations of dayside reconnection signatures

The recent launch of the equatorial spacecraft of the Double Star mission, TC-1, has provided an unprecedented opportunity to monitor the southern hemisphere dayside magnetopause boundary layer in conjunction with northern hemisphere observations by the quartet of Cluster spacecraft. We present first results of one such situation where, on 6 April 2004, both Cluster and the Double Star TC-1 spacecraft were on outbound transits through the dawnside magnetosphere. The observations are consistent with ongoing reconnection on the dayside magnetopause, resulting in a series of flux transfer events (FTEs) seen both at Cluster and TC-1, which appear to lie north and south of the reconnection line, respectively. In fact, the observed polarity and motion of each FTE signature advocates the existence of an active reconnection region consistently located between the positions of Cluster and TC-1, with Cluster observing northward moving FTEs with +/− polarity, whereas TC-1 sees −/+ polarity FTEs. This assertion is further supported by the application of a model designed to track flux tube motion for the prevailing interplanetary conditions. The results from this model show, in addition, that the low-latitude FTE dynamics are sensitive to changes in convected upstream conditions. In particular, changing the interplanetary magnetic field (IMF) clock angle in the model suggests that TC-1 should miss the resulting FTEs more often than Cluster and this is borne out by the observations.

Do online hotel rating schemes influence booking behaviours?

Travelers’ hotel booking behaviors increasingly depend on peers' opinions and online ratings. This study investigates the effects of online hotel ratings on travelers' attitudes toward the hotel and booking intentions, using a 2 × 2 experimental research design. The results suggest that online rating lists are more useful and credible when published by well-known online travel communities (e.g., TripAdvisor). More favorable attitudes toward a hotel and higher booking intentions emerge when the hotel appears in best hotels lists. Finally, for the entries on best hotels lists, better attitudes and higher booking intentions result if the list is published on a well-known online travel community (Tripadvisor), whereas for entries on a worst hotel list, attitudes and booking intentions decrease even further if the list appears on TripAdvisor.

Do online hotel rating schemes influence booking behaviors?

Travelers’ hotel booking behaviors increasingly depend on peers' opinions and online ratings. This study investigates the effects of online hotel ratings on travelers' attitudes toward the hotel and booking intentions, using a 2 × 2 experimental research design. The results suggest that online rating lists are more useful and credible when published by well-known online travel communities (e.g., TripAdvisor). More favorable attitudes toward a hotel and higher booking intentions emerge when the hotel appears in best hotels lists. Finally, for the entries on best hotels lists, better attitudes and higher booking intentions result if the list is published on a well-known online travel community (Tripadvisor), whereas for entries on a worst hotel list, attitudes and booking intentions decrease even further if the list appears on TripAdvisor.