Assessment of cognitive symptoms 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 cognitive symptoms in prHD and early HD individuals.

Assessment of day-to-day functioning 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 in day-to-day activities in prHD and early HD individuals.

Assessment of depression, anxiety and apathy 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 Depression, Anxiety and Apathy in prHD and early HD individuals.

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.

Comparing the fit between BREEAM assessment and design processes

This paper explores the mapping of the environmental assessment process onto design and construction processes. A comparative case study method is used to identify and account for variations in the ‘fit’ between these two processes. The analysis compares eight BREEAM projects (although relevant to LEED, GreenStar, etc.) and distinguishes project-level characteristics and dynamics. Drawing on insights from literature on sustainable construction and assessment methods, an analytic framework is developed to examine the effect of clusters of project and assessment level elements on different types of fit (tight, punctual and bolt-on). Key elements distinguishing between types include: prior working experience with project team members, individual commitment to sustainable construction, experience with sustainable construction, project continuity, project-level ownership of the assessment process, and the nature and continuity of assessor involvement. Professionals with ‘sustainable’ experience used BREEAM judiciously to support their designs (along with other frameworks), but less committed professionals tended to treat it purely as an assessment method. More attention needs to be paid to individual levels of engagement with, and understanding of, sustainability in general (rather than knowledge of technical solutions to individual credits), to ownership of the assessment process and to the potential effect of discontinuities at the project level on sustainable design.

Effects of hydration status on cognitive performance and mood

Although it is well known that water is essential for human homeostasis and survival, only recently have we begun to understand its role in the maintenance of brain function. Herein, we integrate emerging evidence regarding the effects of both dehydration and additional acute water consumption on cognition and mood. Current findings in the field suggest that particular cognitive abilities and mood states are positively influenced by water consumption. The impact of dehydration on cognition and mood is particularly relevant for those with poor fluid regulation, such as the elderly and children. We critically review the most recent advances in both behavioural and neuroimaging studies of dehydration and link the findings to the known effects of water on hormonal, neurochemical and vascular functions in an attempt to suggest plausible mechanisms of action. We identify some methodological weaknesses, including inconsistent measurements in cognitive assessment and the lack of objective hydration state measurements as well as gaps in knowledge concerning mediating factors that may influence water intervention effects. Finally, we discuss how future research can best elucidate the role of water in the optimal maintenance of brain health and function.

Mid-infrared filters for astronomical and remote sensing instrumentation

As improvements to the optical design of spectrometer and radiometer instruments evolve with advances in detector sensitivity, use of focal plane detector arrays and innovations in adaptive optics for large high altitude telescopes, interest in mid-infrared astronomy and remote sensing applications have been areas of progressive research in recent years. This research has promoted a number of developments in infrared coating performance, particularly by placing increased demands on the spectral imaging requirements of filters to precisely isolate radiation between discrete wavebands and improve photometric accuracy. The spectral design and construction of multilayer filters to accommodate these developments has subsequently been an area of challenging thin-film research, to achieve high spectral positioning accuracy, environmental durability and aging stability at cryogenic temperatures, whilst maximizing the far-infrared performance. In this paper we examine the design and fabrication of interference filters in instruments that utilize the mid-infrared N-band (6-15 µm) and Q-band (16-28 µm) atmospheric windows, together with a rationale for the selection of materials, deposition process, spectral measurements and assessment of environmental durability performance.

Treatment of written verb and written sentence production in an individual with aphasia: A clinical study

Background: Although the efficacy of treatments for spoken verb and sentence production deficits in aphasia has been documented widely, less is known about interventions for written verb and written sentence production deficits. Aims: This study documents a treatment aiming to improve production of (a) written subject-verb sentences (involving intransitive verbs) and (b) written subject-verb-object sentences (involving transitive verbs). Methods & Procedures: The participant, a 63-year-old female aphasic speaker, had a marked language comprehension deficit, apraxia of speech, relatively good spelling abilities, and no hemiplegia. The treatment involved intransitive verbs producing subject-verb active sentences and transitive verbs producing subject-verb-object active non-reversible sentences. The treatment was undertaken in the context of current UK clinical practice. Outcomes & Results: Statistical improvements were noted for the trained sets of verbs and sentences. Other improvements were also noted in LW's ability to retrieve some non-treated verbs and construct written sentences. Treatment did not generalise to sentence comprehension and letter spelling to dictation. Conclusions: Our participant's ability to write verbs and sentences improved as a result of the treatment.

The colour, light and contrast manual - designing and managing inclusive built environments

Endorsed by the Society of Light and Lighting, this practical book offers comprehensive guidance on how colour, light and contrast can be incorporated within buildings to enhance their usability. The book provides state-of-the-art, clear guidance as well as a valuable information source for busy professionals involved in the design or management of new and existing environments. The ways colour, light and contrast are used within built environments are critical in determining how people interact with the space, and how confident, safe, and secure they will feel when doing so. They also have a major influence on a person’s sense of well-being and their ability to use the environment independently and without undue effort. Understanding how to use colour and contrast and how they are influenced by both natural and artificial lighting is vital for all those involved in the design and management of the environments and spaces we all use. In recent years there has been a considerable amount of work undertaken to further our understanding of how colour, light and contrast affect emotion and sensory abilities, and how they can assist or hinder people in their everyday lives. Other publications consider these issues individually but The Colour, Light and Contrast Manual: designing and managing inclusive built environments draws knowledge and information together to produce a unique, comprehensive and informative guide to how the three elements can work together to improve the design and management of environments for us all.

The contribution of building research to the independence and quality of life of older people

Many nations are experiencing rapid rises in the life expectancy of their citizens. The implications of this major demographic shift are considerable offering opportunities as well as challenges to reconsider how people should spend their later years. A key task is enhancing the quality of life of older people through enabling them to continue to live independently even though illness, accident or frailty may have severely reduced their physical and sensory abilities and, possibly, mental health. Yet the needs of older people and disabled people have been largely ignored in the design of everyday consumer products, the home, transport systems and the built environment in general. Whilst the need for designers, engineers and technologists to provide products, environments and systems which are inclusive of all members of society is widely accepted, there is little understanding of how this can be achieved. In 1998 the UK Engineering and Physical Sciences Research Council established its EQUAL Initiative. This has encouraged design, engineering and technology researchers in universities to join with their colleagues from the social, medical and health sciences to investigate a wide range of issues experienced by older and disabled people and to propose solutions. Their research, which directly involves older and disabled people and, for example, social housing providers, social services departments, charities, engineering and architectural consultants, and transport firms, has been extremely successful. In a very short time it has influenced government policy on housing, long-term care, and building standards, and findings have been taken up by architects, designers, health-care professionals and bodies which represent older and disabled people.

Educational participation post-16: a longitudinal analysis of intentions and outcomes

The issue of levels of participation in post-compulsory education has been emphasised by the current policy initiatives to increase the age to which some form of participation is compulsory. One of the acknowledged weaknesses of research in the field of children's intentions with regard to participation is the lack of longitudinal data. This paper offers a longitudinal analysis using the Youth Survey from the British Household Panel Survey. The results show that most children can express intentions with regard to future participation very early in their secondary school careers and that these intentions are good predictors of actual behaviour five years later. Intentions to stay on are more consistent than intentions to leave and most children who finally leave at 16 have at some point said they want to remain in education post-16. The strongest association with participation levels is attainment at GCSE. However, there are also influences of gender and parental background and these remain, even after attainment is held constant. The results show the value of focusing on intentions for participation at a very early stage of children's school careers and also the importance of current attempts to reform curriculum and assessment for the 14-19 age group.

Creating teaching and learning in Europe: promoting a new paradigm

The tides of globalization and the unsteady surges and distortions in the evolution of the European Union are causing identities and cultures to be in a state of flux. Education is used by politicians as a major lever for political and social change through micro-management, but it is a crude tool. There can, however, be opportunities within educational experience for individual learners to gain strong, reflexive, multiple identities and multiple citizenship through the engagement of their creative energies. It has been argued that the twenty-first century needs a new kind of creativity characterized by unselfishness, caring and compassion—still involving monetary wealth, but resulting in a healthy planet and healthy people. Creativity and its economically derived relation, innovation, have become `buzz words' of our times. They are often misconstrued, misunderstood and plainly misused within educational conversations. The small-scale pan-European research study upon which this article is founded discovered that more emphasis needs to be placed on creative leadership, empowering teachers and learners, reducing pupils' fear of school, balancing teaching approaches, and ensuring that the curriculum and assessment are responsive to the needs of individual learners. These factors are key to building strong educational provision that harnesses the creative potential of learners, teachers and other stakeholders, values what it is to be human and creates a foundation upon which to build strong, morally based, consistent, participative democracies.

Backward elimination model construction for regression and classification using leave-one-out criteria

A fundamental principle in practical nonlinear data modeling is the parsimonious principle of constructing the minimal model that explains the training data well. Leave-one-out (LOO) cross validation is often used to estimate generalization errors by choosing amongst different network architectures (M. Stone, "Cross validatory choice and assessment of statistical predictions", J. R. Stast. Soc., Ser. B, 36, pp. 117-147, 1974). Based upon the minimization of LOO criteria of either the mean squares of LOO errors or the LOO misclassification rate respectively, we present two backward elimination algorithms as model post-processing procedures for regression and classification problems. The proposed backward elimination procedures exploit an orthogonalization procedure to enable the orthogonality between the subspace as spanned by the pruned model and the deleted regressor. Subsequently, it is shown that the LOO criteria used in both algorithms can be calculated via some analytic recursive formula, as derived in this contribution, without actually splitting the estimation data set so as to reduce computational expense. Compared to most other model construction methods, the proposed algorithms are advantageous in several aspects; (i) There are no tuning parameters to be optimized through an extra validation data set; (ii) The procedure is fully automatic without an additional stopping criteria; and (iii) The model structure selection is directly based on model generalization performance. The illustrative examples on regression and classification are used to demonstrate that the proposed algorithms are viable post-processing methods to prune a model to gain extra sparsity and improved generalization.

Loss of language in early development of autism and specific language impairment

Background:  Several authors have highlighted areas of overlap in symptoms and impairment among children with autism spectrum disorder (ASD) and children with specific language impairment (SLI). By contrast, loss of language and broadly defined regression have been reported as relatively specific to autism. We compare the incidence of language loss and language progression of children with autism and SLI. Methods:  We used two complementary studies: the Special Needs and Autism Project (SNAP) and the Manchester Language Study (MLS) involving children with SLI. This yielded a combined sample of 368 children (305 males and 63 females) assessed in late childhood for autism, history of language loss, epilepsy, language abilities and nonverbal IQ. Results:  language loss occurred in just 1% of children with SLI but in 15% of children classified as having autism or autism spectrum disorder. Loss was more common among children with autism rather than milder ASD and is much less frequently reported when language development is delayed. For children who lost language skills before their first phrases, the phrased speech milestone was postponed but long-term language skills were not significantly lower than children with autism but without loss. For the few who experienced language loss after acquiring phrased speech, subsequent cognitive performance is more uncertain. Conclusions:  Language loss is highly specific to ASD. The underlying developmental abnormality may be more prevalent than raw data might suggest, its possible presence being hidden for children whose language development is delayed.

IQ in children with autism spectrum disorders: data from the Special Needs and Autism Project (SNAP)

Background Autism spectrum disorder (ASD) was once considered to be highly associated with intellectual disability and to show a characteristic IQ profile, with strengths in performance over verbal abilities and a distinctive pattern of ‘peaks’ and ‘troughs’ at the subtest level. However, there are few data from epidemiological studies. Method Comprehensive clinical assessments were conducted with 156 children aged 10–14 years [mean (s.d.)=11.7 (0.9)], seen as part of an epidemiological study (81 childhood autism, 75 other ASD). A sample weighting procedure enabled us to estimate characteristics of the total ASD population. Results Of the 75 children with ASD, 55% had an intellectual disability (IQ<70) but only 16% had moderate to severe intellectual disability (IQ<50); 28% had average intelligence (115>IQ>85) but only 3% were of above average intelligence (IQ>115). There was some evidence for a clinically significant Performance/Verbal IQ (PIQ/VIQ) discrepancy but discrepant verbal versus performance skills were not associated with a particular pattern of symptoms, as has been reported previously. There was mixed evidence of a characteristic subtest profile: whereas some previously reported patterns were supported (e.g. poor Comprehension), others were not (e.g. no ‘peak’ in Block Design). Adaptive skills were significantly lower than IQ and were associated with severity of early social impairment and also IQ. Conclusions In this epidemiological sample, ASD was less strongly associated with intellectual disability than traditionally held and there was only limited evidence of a distinctive IQ profile. Adaptive outcome was significantly impaired even for those children of average intelligence.