Health-related quality of life in Huntington's disease: a comparison of two generic instruments, SF-36 and SIP

Whereas several clinical endpoints in monitoring the response to treatment in patients with Huntington's disease (HD) have been explored, there has been a paucity of research in the quality of life in such patients. The aim of this study was to validate the use of two generic health-related quality of life instruments (the Short Form 36 health survey questionnaire [SF-36] and the Sickness Impact Profile [SIP]) and to evaluate their psychometric properties. We found that both instruments demonstrated acceptable convergent validity and reliability for patients and carers. However, there was an advantage in using the SF-36 because of its more robust construct validity and test-retest reliability; furthermore, motor symptoms appeared to influence some strictly nonmotor dimensions of the SIP. On a pragmatic level, the SF-36 is shorter and quicker to administer and, therefore, easier for patients at various stages of the disease to complete. Thus, the SF-36 would appear to be the recommended instrument of choice for patients with HD and their carers, although further work needs to be done to investigate the sensitivity of this instrument longitudinally. (C) 2004 Movement Disorder Society.

A review of instruments developed to measure outcomes for carers of people with mental health problems

Objective: Community-based care for mental disorders places considerable burden on families and carers. Measuring their experiences has become a priority, but there is no consensus on appropriate instruments. We aimed to review instruments carers consider relevant to their needs and assess evidence for their use. Method: A literature search was conducted for outcome measures used with mental health carers. Identified instruments were assessed for their relevance to the outcomes identified by carers and their psychometric properties. Results: Three hundred and ninety two published articles referring to 241 outcome measures were identified, 64 of which were eligible for review (used in three or more studies). Twenty-six instruments had good psychometric properties; they measured (i) carers' well-being, (ii) the experience of caregiving and (iii) carers' needs for professional support. Conclusion: Measures exist which have been used to assess the most salient aspects of carer outcome in mental health. All require further work to establish their psychometric properties fully.

Divided attention, selective attention and drawing: processing preferences in Williams syndrome are dependent on the task administered

The visuo-spatial abilities of individuals with Williams syndrome (WS) have consistently been shown to be generally weak. These poor visuo-spatial abilities have been ascribed to a local processing bias by some [R. Rossen, E.S. Klima, U. Bellugi, A. Bihrle, W. Jones, Interaction between language and cognition: evidence from Williams syndrome, in: J. Beitchman, N. Cohen, M. Konstantareas, R. Tannock (Eds.), Language, Learning and Behaviour disorders: Developmental, Behavioural and Clinical Perspectives, Cambridge University Press, New York, 1996, pp. 367-392] and conversely, to a global processing bias by others [Psychol. Sci. 10 (1999) 453]. In this study, two identification versions and one drawing version of the Navon hierarchical processing task, a non-verbal task, were employed to investigate this apparent contradiction. The two identification tasks were administered to 21 individuals with WS, 21 typically developing individuals, matched by non-verbal ability, and 21 adult participants matched to the WS group by mean chronological age (CA). The third, drawing task was administered to the WS group and the typically developing (TD) controls only. It was hypothesised that the WS group would show differential processing biases depending on the type of processing the task was measuring. Results from two identification versions of the Navon task measuring divided and selective attention showed that the WS group experienced equal interference from global to local as from local to global levels, and did not show an advantage of one level over another. This pattern of performance was broadly comparable to that of the control groups. The third task, a drawing version of the Navon task, revealed that individuals with WS were significantly better at drawing the local form in comparison to the global figure, whereas the typically developing control group did not show a bias towards either level. In summary, this study demonstrates that individuals with WS do not have a local or a global processing bias when asked to identify stimuli, but do show a local bias in their drawing abilities. This contrast may explain the apparently contrasting findings from previous studies. (C) 2002 Elsevier Science Ltd. All rights reserved.

Perceptual grouping and distance estimates in typical and atypical development: comparing performance across perception, drawing and construction tasks

Perceptual grouping is a pre-attentive process which serves to group local elements into global wholes, based on shared properties. One effect of perceptual grouping is to distort the ability to estimate the distance between two elements. In this study, biases in distance estimates, caused by four types of perceptual grouping, were measured across three tasks, a perception, a drawing and a construction task in both typical development (TD: Experiment 1) and in individuals with Williams syndrome (WS: Experiment 2). In Experiment 1, perceptual grouping distorted distance estimates across all three tasks. Interestingly, the effect of grouping by luminance was in the opposite direction to the effects of the remaining grouping types. We relate this to differences in the ability to inhibit perceptual grouping effects on distance estimates. Additive distorting influences were also observed in the drawing and the construction task, which are explained in terms of the points of reference employed in each task. Experiment 2 demonstrated that the above distortion effects are also observed in WS. Given the known deficit in the ability to use perceptual grouping in WS, this suggests a dissociation between the pre-attentive influence of and the attentive deployment of perceptual grouping in WS. The typical distortion in relation to drawing and construction points towards the presence of some typical location coding strategies in WS. The performance of the WS group differed from the TD participants on two counts. First, the pattern of overall distance estimates (averaged across interior and exterior distances) across the four perceptual grouping types, differed between groups. Second, the distorting influence of perceptual grouping was strongest for grouping by shape similarity in WS, which contrasts to a strength in grouping by proximity observed in the TD participants. (c) 2008 Elsevier Inc. All rights reserved.

Efficacy of three screening instruments in the identification of autistic-spectrum disorders

Background Screening instruments for autistic-spectrum disorders have not been compared in the same sample. Aims To compare the Social Communication Questionnaire (SCQ), the Social Responsiveness Scale (SRS) and the Children's Communication Checklist (CCC). Method Screen and diagnostic assessments on 119 children between 9 and 13 years of age with special educational needs with and without autistic-spectrum disorders were weighted to estimate screen characteristics for a realistic target population. Results The SCQ performed best (area under receiver operating characteristic curve (AUC)=0.90; sensitivity. 6; specificity 0.78). The SRS had a lower AUC (0.77) with high sensitivity (0.78) and moderate specificity (0.67). The CCC had a high sensitivity but lower specificity (AUC=0.79; sensitivity 0.93; specificity 0.46). The AUC of the SRS and CCC was lower for children with IQ < 70. Behaviour problems reduced specificity for all three instruments. Conclusions The SCQ, SRS and CCC showed strong to moderate ability to identify autistic-spectrum disorder in this at-risk sample of school-age children with special educational needs.

Institutional capacity and implementation issues in farmers' rights

Armed with the ‘equity’ and ‘conservation’ arguments that have a deep resonance with farming communities, developing countries are crafting a range of measures designed to protect farmers’ access to innovations, reward their contributions to the conservation and enhancement of plant genetic resources and provide incentives for sustained on-farm conservation. These measures range from the commericialization of farmers’ varieties to the conferment of a set of legally enforceable rights on farming communities – the exercise of which is expected to provide economic rewards to those responsible for on-farm conservation and innovation. The rights-based approach has been the cornerstone of legislative provision for implementing farmers’ rights in most developing countries. In drawing up these measures, developing countries do not appear to have systematically examined or provided for the substantial institutional capacity required for the effective implementation of farmers’ rights provisions. The lack of institutional capacity threatens to undermine any prospect of serious implementation of these provisions. More importantly, the expectation that significant incentives for on-farm conservation and innovation will flow from these ‘rights’ may be based on a flawed understanding of the economics of intellectual property rights. While farmers’ rights may provide only limited rewards for conservation, they may still have the effect of diluting the incentives for innovative institutional breeding programs – with the private sector increasingly relying on non-IPR instruments to profit from innovation. The focus on a rights-based approach may also draw attention away from alternative stewardship-based approaches to the realization of farmers’ rights objectives.

Contrasting the responses of three different ground-based instruments to energetic electron precipitation

In order to make best use of the opportunities provided by space missions such as the Radiation Belt Storm Probes, we determine the response of complementary subionospheric radiowave propagation measurements (VLF), riometer absorption measurements (CNA), and GPS-produced total electron content (vTEC) to different energetic electron precipitation (EEP). We model the relative sensitivity and responses of these instruments to idealised monoenergetic beams of precipitating electrons, and more realistic EEP spectra chosen to represent radiation belts and substorm precipitation. In the monoenergetic beam case, we find riometers are more sensitive to the same EEP event occurring during the day than during the night, while subionospheric VLF shows the opposite relationship, and the change in vTEC is independent. In general, the subionospheric VLF measurements are much more sensitive than the other two techniques for EEP over 200 keV, responding to flux magnitudes two-three orders of magnitude smaller than detectable by a riometer. Detectable TEC changes only occur for extreme monoenergetic fluxes. For the radiation belt EEP case, clearly detectable subionospheric VLF responses are produced by daytime fluxes that are ~10 times lower than required for riometers, while nighttime fluxes can be 10,000 times lower. Riometers are likely to respond only to radiation belt fluxes during the largest EEP events and vTEC is unlikely to be significantly disturbed by radiation belt EEP. For the substorm EEP case both the riometer absorption and the subionospheric VLF technique respond significantly, as does the change in vTEC, which is likely to be detectable at ~3-4 TECu.

In-situ comparison of the NOy instruments flown in MOZAIC and SPURT

Two aircraft instruments for the measurement of total odd nitrogen (NOy) were compared side by side aboard a Learjet A35 in April 2003 during a campaign of the AFO2000 project SPURT (Spurengastransport in der Tropopausenregion). The instruments albeit employing the same measurement principle (gold converter and chemiluminescence) had different inlet configurations. The ECO-Physics instrument operated by ETH-Zürich in SPURT had the gold converter mounted outside the aircraft, whereas the instrument operated by FZ-Jülich in the European project MOZAIC III (Measurements of ozone, water vapour, carbon monoxide and nitrogen oxides aboard Airbus A340 in-service aircraft) employed a Rosemount probe with 80 cm of FEP-tubing connecting the inlet to the gold converter. The NOy concentrations during the flight ranged between 0.3 and 3 ppb. The two data sets were compared in a blind fashion and each team followed its normal operating procedures. On average, the measurements agreed within 7%, i.e. within the combined uncertainty of the two instruments. This puts an upper limit on potential losses of HNO3 in the Rosemount inlet of the MOZAIC instrument. Larger transient deviations were observed during periods after calibrations and when the aircraft entered the stratosphere. The time lag of the MOZAIC instrument observed in these instances is in accordance with the time constant of the MOZAIC inlet line determined in the laboratory for HNO3.