Background Amyotrophic lateral sclerosis (ALS) is a progressive debilitating neurodegenerative disease, with a life expectancy of 3-5 years from first symptom. There is compelling evidence that those who attend a multidisciplinary clinic experience improved survival. The purpose of the study was to explore the survival of patients with ALS ascertained through population-based Registers in the Republic of Ireland (RoI) and Northern Ireland (NI), and to determine whether centralisation of services confers advantage compared with communitybased care supported by a specialist care worker. Methods The island of Ireland is divided into two countries, RoI and NI, each with an independent healthcare system. Both countries have population-based ALS Registers with full ascertainment. Data from all 719 incident ALS cases from Ireland and NI, diagnosed between 1 January 2005 and 31 December 2010, were used in the analysis. Results A survival benefit was identified for patients who attended the multidisciplinary ALS clinic in the RoI. (HR 0.59, 95% CI 0.49 to 0.71, p<0.001). This difference was preserved following multivariate analysis. A trend towards improved survival was noted for patients with ALS from NI when compared with RoI patients who did not attend a multidisciplinary clinic. Conclusions Centralised multidisciplinary care confers a survival advantage for patients with ALS and is superior to devolved community-based care. We propose that multiple decision-making processes within a multidisciplinary setting lead to an enriched set of clinical encounters for the patient and carer that enhances clinical outcome.

Population-based disease registers identify and characterize all cases of disease, including those that might otherwise be neglected. Prospective population-based registers in neurodegeneration are necessary to provide comprehensive data on the whole phenotypic spectrum and can guide planning of health services. With the exception of the rare disease amyotrophic lateral sclerosis, few complete population-based registers exist for neurodegenerative conditions. Incomplete ascertainment, limitations and uncertainty in diagnostic categorization, and failure to recognize sources of bias reduce the accuracy and usefulness of many registers. Common biases include population stratification, the use of prevalent rather than incident cases in earlier years, changes in disease understanding and diagnostic criteria, and changing demographics over time. Future registers are at risk of funding shortfalls and changes to privacy legislation. Notwithstanding, as heterogeneities of clinical phenotype and disease pathogenesis are increasingly recognized in the neurodegenerations, well-designed longitudinal population-based disease registers will be an essential requirement to complete clinical understanding of neurodegenerative diseases.

Background It is postulated that orthostatic hypotension ( OH ), a reduction in blood pressure (≥20/10 mm Hg) within 3 minutes of standing, may increase cognitive decline because of cerebral hypoperfusion. This study assesses the impact of OH on global cognition at 4‐year follow‐up, and the impact of age and hypertension on this association. Methods and Results Data from waves 1 and 3 of TILDA (The Irish Longitudinal Study on Ageing) were used. Baseline blood pressure response to active stand was assessed using beat‐to‐beat blood pressure monitoring. Two measures of OH were used—at 40 seconds ( OH 40) and 110 seconds ( OH 110). Global cognition was measured using the Montreal Cognitive Assessment. Mixed‐effects Poisson regression assessed whether baseline OH was associated with a decline in global cognition at 4‐year follow‐up. The analysis was repeated, stratifying by age (age 50–64 years and age ≥65 years), and including an interaction between OH and hypertension. Baseline OH 110 was associated with an increased error rate in Montreal Cognitive Assessment at follow‐up (incident rate ratio 1.17, P =0.028). On stratification by age, the association persists in ages 50 to 64 years (incident rate ratio 1.25, P =0.048), but not ages ≥65 years. Including an interaction with hypertension found those with co‐existent OH 110 and hypertension (incident rate ratio 1.27, P =0.011), or OH 40 and hypertension (incident rate ratio 1.18, P =0.017), showed an increased error rate; however, those with isolated OH 110, OH 40, or isolated hypertension did not. Conclusions OH is associated with a decline in global cognition at 4‐year follow‐up, and this association is dependent on age and co‐existent hypertension.

Background Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative condition with a mean life expectancy of 3 years from first symptom. Understanding the factors that are important to both patients and their caregivers has the potential to enhance service delivery and engagement, and improve efficiency. The Discrete Choice Experiment (DCE) is a stated preferences method which asks service users to make trade-offs for various attributes of health services. This method is used to quantify preferences and shows the relative importance of the attributes in the experiment, to the service user. Methods A DCE with nine choice sets was developed to measure the preferences for health services of ALS patients and their caregivers and the relative importance of various aspects of care, such as timing of care, availability of services, and decision making. The DCE was presented to patients with ALS, and their caregivers, recruited from a national multidisciplinary clinic. A random effects probit model was applied to estimate the impact of each attribute on a participant’s choice. Results Patients demonstrated the strongest preferences about timing of receiving information about ALS. A strong preference was also placed on seeing the hospice care team later rather than early on in the illness. Patients also indicated their willingness to consider the use of communication devices. Grouping by stage of disease, patients who were in earlier stages of disease showed a strong preference for receipt of extensive information about ALS at the time of diagnosis. Caregivers showed a strong preference for engagement with healthcare professionals, an attribute that was not prioritised by patients. Conclusions The DCE method can be useful in uncovering priorities of patients and caregivers with ALS. Patients and caregivers have different priorities relating to health services and the provision of care in ALS, and patient preferences differ based on the stage and duration of their illness. Multidisciplinary teams must calibrate the delivery of care in the context of the differing expectations, needs and priorities of the patient/caregiver dyad.

In a patient cohort with relatively preserved cognitive function (65%), anxiety and depression at Time 1, as measured by the HADS, were the best predictors of caregiver burden at Time 3. This observation provides a mechanism by which caregiver burden can be identified by health-care professionals and a stepped care programme of intervention initiated.

Article:Tobin, K, Gilthorpe, MS orcid.org/0000-0001-8783-7695, Rooney, J et al. (4 more authors) (2016) Age-period-cohort analysis of trends in amyotrophic lateral sclerosis incidence. Journal of Neurology, 263 (10). pp. 1919-1926 https://doi.org/10.1007/s00415-016-8215-z © 2016, Springer-Verlag Berlin Heidelberg. This is an author produced version of a paper published in Journal of Neurology. Uploaded in accordance with the publisher's self-archiving policy.The final publication is available at Springer via http://dx.doi.org/10.1007/s00415-016-8215-z eprints@whiterose.ac.uk https://eprints.whiterose.ac.uk/ Reuse Unless indicated otherwise, fulltext items are protected by copyright with all rights reserved. The copyright exception in section 29 of the Copyright, Designs and Patents Act 1988 allows the making of a single copy solely for the purpose of non-commercial research or private study within the limits of fair dealing. The publisher or other rights-holder may allow further reproduction and re-use of this version -refer to the White Rose Research Online record for this item. Where records identify the publisher as the copyright holder, users can verify any specific terms of use on the publisher's website. TakedownIf you consider content in White Rose Research Online to be in breach of UK law, please notify us by emailing eprints@whiterose.ac.uk including the URL of the record and the reason for the withdrawal request.

This study demonstrates that two-thirds of depressed older people are not prescribed antidepressant/antipsychotic therapy. It is important to raise awareness of depression among older people and healthcare professionals, with particular focus on the fact that LLD is not an inevitable consequence of ageing and effective treatment is available.

Objectives: We aimed to explore the relationship between religiosity and depressive symptoms longitudinally. Method: We used four waves (2009–2016) of the Irish Longitudinal Study on Ageing (TILDA) to create growth curve models (GCM) of depressive symptoms and religious attendance/importance in a sample aged 50+ in Ireland and structural models to assess the longitudinal associations between religious attendance/importance and depressive symptoms. We tested whether this relationship was mediated by social connectedness. Results: GCM showed that higher religious attendance at baseline was associated with lower baseline depressive symptoms, while higher religious importance was associated with higher baseline depressive symptoms. Social connectedness partially mediated the baseline associations between religious attendance and lower depressive symptoms. There were no associations between religious factors and the development of depressive symptoms over time. Discussion: This study found that the relationship between religion and depressive symptoms is complex, and any protective effect was driven by religious attendance.