Contributions of Caregiver Management Styles to the Discrepancy Between Reported and Observed Task Performance in People with Dementia

Background: The identification and understanding of the discrepancy between caregivers’ reports of people with dementia’s (PwD) performance of activities of daily living (ADLs) and observed performance, could clarify what kind of support a PwD effectively needs when completing tasks. Strategies used by caregivers have not been included in the investigation of this discrepancy. Objectives: To (1) investigate if caregivers’ report of PwD’s ADL performance are consistent with PwD’s observed performance; (2) explore if caregiver management styles, depression, and anxiety, contribute to this discrepancy. Methods: PwD (n=64) were assessed with standardised performance-based (AMPS) and informant-based (DAD) ADL assessments. Caregivers completed depression (PHQ-9), anxiety (GAD-7), and dementia management style (DMSS: criticism, active-management, and encouragement) questionnaires. Cohen’s kappa determined agreement/disagreement in ADL performance. To investigate the potential discrepancy between the DAD and AMPS, a continuous variable was generated: comparative ADL score. Multiple linear regression analysis explored whether caregivers’ management styles, depression or anxiety could explain the ADL discrepancy. Results: Poor level of agreement between observed and reported ADL performance [k= -0.025 (95%CI -0.123 – 0.073)] was identified, with most caregivers underestimating ADL performance. The combined model explained 18% (R2=0.18, F(5, 55)=2.52,p=<0.05) of the variance of the comparative ADL score. Active-management (β=-0.037,t(60)=-3.363, p=0.001) and encouragement (β=0.025,t(60)=2.018, p=0.05) styles made the largest and statistically significant contribution to the model. Conclusions: Encouragement style could be advised for caregivers who underestimate ADL performance, while active management style for those who overestimate it. Findings have scope to increase caregivers’ abilities to support PwD activity engagement in daily life

A New and Tidier Setting How Does Environmental Clutter Affect People With Dementia’s Ability to Perform Activities of Daily Living?

Background: The relationship between the physical environment and the person with dementia’s (PwD) activities of daily living (ADLs) task performance is controversial. Although the general assumption is that this population benefits from their home environment when performing ADLs, very few experimental studies have been conducted to date. Objectives: The aim was to investigate the influence of the environment (home vs. Research-lab) and the role of clutter on ADL performance. Methods: Sixty-five PwD were evaluated with a performance-based ADL assessment (at home and clutter-free Research-lab). Paired t tests compared ADL performance and level of clutter in both environments. Multiple regression analysis investigated factors associated with better ADL performance. Results: Overall, PwD performed better at home even though clutter was significantly lower in the Research-lab. When stratified by dementia stage, PwD in the moderate stage of the disease performed better at home. Conclusion: Absence of clutter in the Research-Lab did not appear to play a beneficial role in ADLs. When stratified by dementia stage, only PwD in the moderate stage appeared to benefit from their home environment when performing ADL tasks. Future studies are required to elucidate the wider role of the environment in supporting engagement in daily activities in different dementia stages

Decentralised energy futures: The changing emissions reduction landscape

© 2015 The Authors. Published by Elsevier B.V. The world is witnessing an energy revolution as renewables become more competitive and energy security becomes a high priority for an increasing number of countries. This development is changing the point along the supply chain ripe for reducing emissions. Whereas carbon capture and storage (CCS) coupled to coal or gas power production offers the potential to decarbonise the current centralised power systems, this relies on a significant increase in electrification to achieve deep emission reductions beyond the power sector, including industrial emissions and transportation. At the same time there is a trend towards decentralised industrial processes, e.g., driven by cost reductions in decentralised production systems and miniature processing plant. New strategies for reducing emissions from decentralised industrial and energy emission point sources will be increasingly important. This paper evaluates different emission reduction strategies that may be relevant to a decentralised energy and manufacturing future, including increased electrification, energy storage, renewable energy and renewable feedstock. Systemic opportunities or barriers and considerations of policy and decentralised decision-making are examined

Non-verbal IQ Gains from Relational Operant Training Explain Variance in Educational Attainment: An Active-Controlled Feasibility Study

Research suggests that training relational operant patterns of behavior can lead to increases in general cognitive ability and educational outcomes. Most studies to date have been under-powered and included proxy measures of educational attainment. We attempted to extend previous findings with increased experimental control in younger children (aged 6.9–10.1 years). Participants (N = 49) were assigned to either a relational training or chess control group. Over 5 months, teachers assigned class time to complete either relational training or play chess. Those who were assigned relational training gained 8.9 non-verbal IQ (NVIQ) points, while those in the control condition recorded no gains (dppc2 = .99). Regression analyses revealed that post-training NVIQ predicted reading test scores (conducted approximately 1 month later) over and above baseline NVIQ in the experimental condition only, consistent with what we might expect in a full test of far transfer towards educational outcomes

Mutations in TOP3A Cause a Bloom Syndrome-like Disorder

Bloom syndrome, caused by biallelic mutations in BLM, is characterized by prenatal-onset growth deficiency, short stature, an erythematous photosensitive malar rash, and increased cancer predisposition. Diagnostically, a hallmark feature is the presence of increased sister chromatid exchanges (SCEs) on cytogenetic testing. Here, we describe biallelic mutations in TOP3A in ten individuals with prenatal-onset growth restriction and microcephaly. TOP3A encodes topoisomerase III alpha (TopIIIα), which binds to BLM as part of the BTRR complex, and promotes dissolution of double Holliday junctions arising during homologous recombination. We also identify a homozygous truncating variant in RMI1, which encodes another component of the BTRR complex, in two individuals with microcephalic dwarfism. The TOP3A mutations substantially reduce cellular levels of TopIIIα, and consequently subjects’ cells demonstrate elevated rates of SCE. Unresolved DNA recombination and/or replication intermediates persist into mitosis, leading to chromosome segregation defects and genome instability that most likely explain the growth restriction seen in these subjects and in Bloom syndrome. Clinical features of mitochondrial dysfunction are evident in several individuals with biallelic TOP3A mutations, consistent with the recently reported additional function of TopIIIα in mitochondrial DNA decatenation. In summary, our findings establish TOP3A mutations as an additional cause of prenatal-onset short stature with increased cytogenetic SCEs and implicate the decatenation activity of the BTRR complex in their pathogenesis

Acceptance and commitment therapy for people living with motor neuron disease: an uncontrolled feasibility study

Background Motor neuron disease (MND) is a fatal, progressive neurodegenerative disease that causes progressive weakening and wasting of limb, bulbar, thoracic and abdominal muscles. Clear evidence-based guidance on how psychological distress should be managed in people living with MND (plwMND) is lacking. Acceptance and Commitment Therapy (ACT) is a form of psychological therapy that may be particularly suitable for this population. However, to the authors' knowledge, no study to date has evaluated ACT for plwMND. Consequently, the primary aim of this uncontrolled feasibility study was to examine the feasibility and acceptability of ACT for improving the psychological health of plwMND. Methods PlwMND aged ≥ 18 years were recruited from 10 UK MND Care Centres/Clinics. Participants received up to 8 one-to-one ACT sessions, developed specifically for plwMND, plus usual care. Co-primary feasibility and acceptability outcomes were uptake (≥ 80% of the target sample [N = 28] recruited) and initial engagement with the intervention (≥ 70% completing ≥ 2 sessions). Secondary outcomes included measures of quality of life, anxiety, depression, disease-related functioning, health status and psychological flexibility in plwMND and quality of life and burden in caregivers. Outcomes were assessed at baseline and 6 months. Results Both a priori indicators of success were met: 29 plwMND (104%) were recruited and 76% (22/29) attended ≥ 2 sessions. Attrition at 6-months was higher than anticipated (8/29, 28%), but only two dropouts were due to lack of acceptability of the intervention. Acceptability was further supported by good satisfaction with therapy and session attendance. Data were possibly suggestive of small improvements in anxiety and psychological quality of life from baseline to 6 months in plwMND, despite a small but expected deterioration in disease-related functioning and health status. Conclusions There was good evidence of acceptability and feasibility. Limitations included the lack of a control group and small sample size, which complicate interpretation of findings. A fully powered RCT to evaluate the clinical and cost-effectiveness of ACT for plwMND is underway

Biallelic C1QBP Mutations Cause Severe Neonatal-, Childhood-, or Later-Onset Cardiomyopathy Associated with Combined Respiratory-Chain Deficiencies

Complement component 1 Q subcomponent-binding protein (C1QBP; also known as p32) is a multi-compartmental protein whose precise function remains unknown. It is an evolutionary conserved multifunctional protein localized primarily in the mitochondrial matrix and has roles in inflammation and infection processes, mitochondrial ribosome biogenesis, and regulation of apoptosis and nuclear transcription. It has an N-terminal mitochondrial targeting peptide that is proteolytically processed after import into the mitochondrial matrix, where it forms a homotrimeric complex organized in a doughnut-shaped structure. Although C1QBP has been reported to exert pleiotropic effects on many cellular processes, we report here four individuals from unrelated families where biallelic mutations in C1QBP cause a defect in mitochondrial energy metabolism. Infants presented with cardiomyopathy accompanied by multisystemic involvement (liver, kidney, and brain), and children and adults presented with myopathy and progressive external ophthalmoplegia. Multiple mitochondrial respiratory-chain defects, associated with the accumulation of multiple deletions of mitochondrial DNA in the later-onset myopathic cases, were identified in all affected individuals. Steady-state C1QBP levels were decreased in all individuals' samples, leading to combined respiratory-chain enzyme deficiency of complexes I, III, and IV. C1qbp(-/-) mouse embryonic fibroblasts (MEFs) resembled the human disease phenotype by showing multiple defects in oxidative phosphorylation (OXPHOS). Complementation with wild-type, but not mutagenized, C1qbp restored OXPHOS protein levels and mitochondrial enzyme activities in C1qbp(-/-) MEFs. C1QBP deficiency represents an important mitochondrial disorder associated with a clinical spectrum ranging from infantile lactic acidosis to childhood (cardio)myopathy and late-onset progressive external ophthalmoplegia.This study was supported by the German BMBF and Horizon2020 through E-Rare project GENOMIT (01GM1603 and 01GM1207 to H.P.; FWF-I 2741-B26 to J.A.M.); Vereinigung zur Förderung Pädiatrischer Forschung Salzburg; EU FP7 MEET Project (317433 to H.P. and J.A.M.); Horizon2020 Project SOUND (633974 to H.P.); Marie Skłodowska-Curie Actions Reintegration Fellowship (Mitobiopath-705560 to C.G.); UK NHS Highly Specialised Mitochondrial Service (R.W.T.); Wellcome Centre for Mitochondrial Research (203105/Z/16 to Z.M.C.-L., R.N.L., and R.W.T.); MRC Centre for Neuromuscular Diseases (G0601943 to R.W.T. and P.F.C.); Lily Foundation (R.W.T. and K.T.); UK NIHR fellowship (NIHR-HCS-D12-03-04 to C.L.A.); Wellcome Senior Fellowship (101876/Z/13/Z to P.F.C.); UK NIHR award and MRC Mitochondrial Biology Unit (MC_UP_1501/2 to P.F.C.); NIH (R01 GM0077465 and R35 GM122455 to V.K.M.); EMBO fellowship (ALTF 554-2015 to A.A.J.); UK MRC core funding for the Mitochondrial Biology Unit of the University of Cambridge (MC_U105697135 to A.R.D., P.R.G., and M. Minczuk); Portuguese Fundação para a Ciência e a Tecnologia (PD/BD/105750/2014 to P.R.G.); Italian Telethon (GSP16001 to G.P.C.); Fondazione Cariplo (2014-1010 to D.R.); Strategic Research Center in Private Universities from MEXT; and Practical Research Project for Rare/Intractable Diseases from AMED

Pheromones and Other Semiochemicals for Monitoring Rare and Endangered Species

As global biodiversity declines, biodiversity and conservation have become ever more important research topics. Research in chemical ecology for conservation purposes has not adapted to address this need. During the last 10-15 years, only a few insect pheromones have been developed for biodiversity and conservation studies, including the identification and application of pheromones specifically for population monitoring. These investigations, supplemented with our knowledge from decades of studying pest insects, demonstrate that monitoring with pheromones and other semiochemicals can be applied widely for conservation of rare and threatened insects. Here, I summarize ongoing conservation research, and outline potential applications of chemical ecology and pheromone-based monitoring to studies of insect biodiversity and conservation research. Such applications include monitoring of insect population dynamics and distribution changes, including delineation of current ranges, the tracking of range expansions and contractions, and determination of their underlying causes. Sensitive and selective monitoring systems can further elucidate the importance of insect dispersal and landscape movements for conservation. Pheromone-based monitoring of indicator species will also be useful in identifying biodiversity hotspots, and in characterizing general changes in biodiversity in response to landscape, climatic, or other environmental changes