Survival of Huntington’s disease patients in Serbia: longer survival in female patients

The objective of this study was to estimate probability of survival of Huntington’s disease (HD) patients in Serbia as a function of CAG repeat length and selected demographic variables. This follow-up study was carried out at the Institute of Neurology, Clinical Centre of Serbia, Belgrade, 1982–2004. The study group consisted of 112 HD patients. The significant inverse correlation was found between CAG repeat length and age at onset of HD (r = −0.732, P = 0.001) and age at death (r = −0.760, P = 0.001). The cumulative probabilities of survival in a five, ten, fifteen, and twenty-years’ period were 90.9, 63.2, 10.3 and 4.5%, respectively. Higher survival probabilities were registered in female patients, as well as in those with older age at onset and lower number of CAG repeat length (≤46). The Cox regression analysis showed that significantly poorer outcome of HD in our population was related to younger age at onset (HR-hazard ratio = 1.9; P = 0.047), and larger CAG numbers (HR = 2.4; P = 0.071). The female sex was statistically significantly associated with longer survival (HR = 0.4; P = 0.007). These data might be of some importance for further exploration of natural history and prognosis of HD.     

Perception of Alzheimer’s disease in the French population

Background: Objective

The aim of this study was to investigate the perception, knowledge, opinions and beliefs about AD in the French population to improve care for patients with Alzheimer’s disease.

Design

A cross-sectional telephone survey in 2008.

Setting

French “Alzheimer Plan 2008–2012”.

Participants

2013 respondents, representative of the French population.

Measurements

The respondents answered a questionnaire in which they were presented with a series of attitudinal statements about health in general and AD.

Results

Main results are summarized as followed: (a) Respondents think that AD can be devastating for a family (93%). This devastating feeling prevails in the population >75 years old and in people who know patient with AD. (b) General population has a reasonable knowledge about AD, but is still unable to recognize early stages: 95% of respondents considered that difficulties to manage administrative papers and find their way back home suggest early AD. (c) 91% of the population would like to know the diagnosis if they had AD. (d) 38% of the population know or have known at least one patient within their social circle. People think that the care of AD’s patient can be better but they trust in the French government to improve it.

Conclusion

General representation of AD is changing in a positive way and, even though AD is perceived as a calamity, people are confident that solutions will be found in the future.     

Dietary fibre and cardiovascular disease mortality in the UK Women’s Cohort Study

Dietary fibre has been associated with improvements in key risk factors for cardiovascular disease (CVD). Prior research has focussed more on CVD development in men and our aim was therefore to explore the association between dietary fibre intake and CVD mortality using data from the United Kingdom Women’s Cohort Study (UKWCS). Dietary fibre intake from 31,036 women was calculated both as non-starch polysaccharide (NSP) and using the Association of Official Analytical Chemist (AOAC) method from food-frequency questionnaires. Participants were free from history of CVD at baseline and mean age at recruitment was 51.8 years (standard deviation 9.2). Mortality records for participants were linked from national registry data and 258 fatal CVD cases [130 stroke, 128 coronary heart disease (CHD)] were observed over an average follow-up period of 14.3 years. Total dietary fibre (NSP/AOAC) and fibre from different food sources were not associated with fatal CHD, stroke or CVD risk in the full sample. For every 6 g/day increase in NSP, the hazard ratio (HR) was 0.91 (95 % confidence interval (CI) 0.76–1.08) or for every 11 g/day increase in fibre assessed as AOAC, the HR was 0.92 (95 % CI 0.80–1.05). Sensitivity analyses suggest a possible protective association for cereal sources of fibre on fatal stroke risk in overweight women, HR 0.80 (95 % CI 0.65–0.93) p < 0.01. In the UKWCS, a sample of health-conscious women, greater dietary fibre intake may confer no additional cardiovascular benefit, in terms of mortality, but may contribute to lower fatal stroke risk in some subgroups such as overweight women.     

Progress in the development of new drugs in Alzheimer’s disease

Alzheimer’s disease (AD) is an age-related neurodegenerative disease with a global prevalence estimated at 26.55 million in 2006. During the past decades, several agents have been approved that enhance cognition of AD patients. However, the effectiveness of these treatments are limited or controversial and they do not modify disease progression. Recent advances in understanding AD pathogenesis have led to the development of numerous compounds that might modify the disease process. AD is mainly characterized neuropathologically by the presence of two kinds of protein aggregates: extracellular plaques of Abeta-peptide and intracellular neurofibrillary tangles. Abeta and tau could interfere in an original way contributing to a cascade of events leading to neuronal death and transmitter deficits. Investigation for novel therapeutic approaches targeting the presumed underlying pathogenic mechanisms is major focus of research. Antiamyloid agents targeting production, accumulation, clearance, or toxicity associated with Abeta peptide, are some approaches under investigation to limit extracellular plaques of Abeta-peptide accumulation. We can state as an example: Abeta passive and active immunization, secretases modulation, Abeta degradation enhancement, or antiaggregation and antifibrillization agents. Tau-related therapies are also under clinical investigation but few compounds are available. Another alternative approach under development is neuroprotective agents such as antioxidants, anti-inflammatory drugs, compounds acting against glutamate mediated neurotoxicity. Neurorestorative approaches through neurotrophin or cell therapy also represent a minor avenue in AD research. Finally, statins, receptor for advanced glycation end products inhibitors, thiazolidinediones, insulin, and hormonal therapies are some other ways of research for a therapeutic approach of Alzheimer’s disease. Taking into account AD complexity, it becomes clear that polypharmacology with drugs targeting different sites could be the future treatment approach and a majority of the recent drugs under evaluation seems to act on multiple targets. This article exposes general classes of disease-modifying therapies under investigation.     

Long-term medical costs of Alzheimer’s disease: matched cohort analysis

The European Journal of Health Economics - Medical costs associated with Alzheimer’s disease (AD) are characterised by uncertainty and are&nbsp;often presented in a format unsuitable for...     

Pulmonary embolus and maternal mortality in the antenatal period--evidence of a trend?

An analysis of pulmonary embolus mortality data was undertaken to investigate a putative change in maternal mortality due to pulmonary embolism in the antenatal period of pregnancy. A comparison was made of mortality due to pulmonary embolus for pregnant and non-pregnant women of reproductive age in England and Wales in the four triennia from 1976. During this period the antenatal pulmonary embolus mortality rate remained unchanged, whereas a statistically significant decline in non-pregnancy related pulmonary embolus mortality was observed. A comparison of the mortality trends between the two groups found the difference to be statistically highly significant. These findings strongly suggest that, over the twelve years investigated, women in the antenatal period of pregnancy have not benefited from the reduced risk of death from pulmonary embolus enjoyed by their non-pregnant counterparts.     

‘All the burden on all the carers’: exploring quality of life with family caregivers of Huntington’s disease patients

Background

Existing research suggests that family caregivers of persons with Huntington??s disease face a unique series of problems, linked to the complex nature of the disease. There is little research that explicitly investigates the impact of HD on the quality of life (QoL) of the family caregiver. The purpose of this study was to explore the quality of life issues for family carers of Huntington??s disease patients in a focus group setting.

Methods

Participants were recruited via a Huntington??s Disease Association (HDA) family conference day. Six semi-directed focus groups (n?=?47) explored disease-specific aspects of QoL that were deemed important to family carers of this carer group. Data were analysed using Interpretative Phenomenological Analysis (IPA).

Results

Analysis of the focus group data identified four superordinate themes: ??Levels of Support??, ??Dissatisfaction with Caregiving Role??, ??Practical Aspects of Caring?? and ??Feelings and Emotional Well-being??.

Conclusions

These data provide evidence that QoL is compromised in many ways for HD family carers. The carers in this study often negated their own needs as their caregiving role overwhelmed them and ??took over?? their lives.     

Validation of the Japanese translation of the Swallowing Disturbance Questionnaire in Parkinson’s disease patients

Purpose

The Swallowing Disturbance Questionnaire (SDQ) was developed as a self-rated screening tool for dysphagia in patients with Parkinson??s disease (PD). We developed the Japanese version of this questionnaire (SDQ-J), according to the cross-cultural adaptation guidelines, and examined its reliability.

Methods

Subjects were 61 Japanese patients with PD (mean age, 67.0?±?9.2?years) who answered the SDQ-J before undergoing videofluoroscopic examination of swallowing (VF). We compared the findings of the questionnaire with the patients?? aspiration status during VF.

Results

Cronbach??s alpha coefficient for the 15 questions of the SDQ-J was 0.84. According to the SDQ-J, 15 patients (24.6%) were diagnosed with dysphagia, while 9 patients (14.8%) aspirated liquid during VF. The sensitivity and specificity of the SDQ-J in predicting aspiration were 77.8 and 84.6%, respectively; therefore, the SDQ-J significantly predicted aspiration during VF (P?Conclusions The SDQ-J appears to be a reliable and useful screening tool for Japanese PD patients with aspiration. As the NPV was higher than the PPV in the SDQ-J, this questionnaire could potentially be used for early identification of severe dysphagia in patients with PD.     

Quality of life of caregivers in Parkinson’s disease

Objective: To assess the impact of PD on informal caregivers of patients and identify the main factors related to caregiver strain. Patients and methods: Pairs of PD patients and their caregivers. Evaluation by neurologists included the Hoehn and Yahr, Schwab and England, UPDRS (parts 1–3), ISAPD, and Pfeiffers SPMSQ rating scales. Patients completed the Euro-QoL 5D, PDQ-8, and Hospital Anxiety and Depression Scale. The SQLC was used to assess caregivers quality of life (QoL), with caregivers, in turn, applying the Euro-QoL and PDQ-8 to assess patients health-related quality of life (HRQoL). Multiple linear regression models were fitted to ascertain factors linked to the SQLC. Results: Significant correlations were in evidence between the following scores: SQLC and clinical rating scales and SQLC and patients HRQoL. Based on multiple regression analysis, patients functional state (ADL) proved to be the main predictor of caregivers QoL. Self- and caregiver-assessed patients HRQoL also proved to be a relevant factor. Conclusions: (1) Patients functional state was significantly related to caregivers psychosocial burden; (2) patients HRQoL proved to be an additional factor linked to caregiver QoL; (3) improvement of patient disability and HRQoL might alleviate caregiver strain.     

Relative contributions of biomarkers in Alzheimer’s disease

PurposeTo assess relationships between biomarkers for Alzheimer’s disease (AD) and their potential contributions to AD.MethodsBiomarkers and cognitive evaluations were assessed longitudinally in 179 patients with mild cognitive impairment, from the Alzheimer’s Disease Neuroimaging Initiative from 2003 to 2006, and were used to examine, at any given time, the joint contributions of hippocampal volume, whole brain volume, and brain glucose metabolism on clinical AD progression, using the Alzheimer’s Disease Assessment Scale-Cognitive subscale (ADAS-Cog). Marginal structural models were applied, and an inverse-probability of treatment weight estimation was used to account for time-dependent confounding between study variables.ResultsAt any given time, population-level differences (e.g., 1-standard deviation [SD] increase) in brain glucose metabolism (?1.036; 95% confidence interval [95% CI], ?1.608, ?0.464) and hippocampal volume (–1.537; 95% CI, ?2.399, ?0.674) independently reduced mean (ADAS-Cog), whereas a 1-SD increase in whole brain volume did not (0.372; 95% CI, ?0.283, 1.027). The effects of brain glucose metabolism differed in subgroups defined by baseline covariates (e.g., age), but no subgroup effects were observed for hippocampal volume and brain volume.ConclusionsBrain glucose metabolism and hippocampal volume represent relevant biological markers in subjects at risk for AD.     

Direct costs of Alzheimer’s disease in Germany

Objectives  

The aim of this analysis was to determine the direct costs for patients with Alzheimer’s disease (AD) based on data supplied by a large German statutory health insurance (BARMER). Focus of the present evaluation is the exposure of total direct costs, survival rates and the types of care distribution of patients with AD.     

Aspirin in the primary prevention of cardiovascular disease in the Women’s Health Study: Effect of noncompliance

Randomized evidence for aspirin in the primary prevention of cardiovascular disease (CVD) among women is limited and suggests at most a modest effect for total CVD. Lack of compliance, however, can null-bias estimated effects. We used marginal structural models (MSMs) to estimate the etiologic effect of continuous aspirin use on CVD events among 39,876 apparently healthy female health professionals aged 45 years and older in the Women's Health Study, a randomized trial of 100 mg aspirin every other day versus placebo. As-treated analyses and MSMs controlled for time-varying determinants of aspirin use and CVD. Predictors of aspirin use differed by randomized group and prior use and included medical history, CVD risk factors, and intermediate CVD events. Previously reported intent-to-treat analyses found small non-significant effects of aspirin on total CVD (hazard ratio (HR) = 0.91, 95 % confidence interval (CI) = 0.81-1.03) and CVD mortality (HR = 0.95, 95 % CI = 0.74-1.22). As-treated analyses were similar for total CVD with a slight reduction in CVD mortality (HR = 0.88, 95 % CI = 0.67-1.16). MSMs, which adjusted for non-compliance, were similar for total CVD (HR = 0.93; 95 % CI: 0.81, 1.07) but suggested lower CVD mortality with aspirin use (HR = 0.76; 95 % CI: 0.54, 1.08). Adjusting for non-compliance had little impact on the estimated effect of aspirin on total CVD, but strengthened the effect on CVD mortality. These results support a limited effect of low-dose aspirin on total CVD in women, but potential benefit for CVD mortality.     

Agricultural activities and the incidence of Parkinson’s disease in the general French population

Most studies on pesticides and Parkinson’s disease (PD) focused on occupational exposure in farmers. Whether non-occupational exposure is associated with PD has been little explored. We investigated the association between agricultural characteristics and PD incidence in a French nationwide ecologic study. We hypothesized that persons living in regions with agricultural activities involving more intensive pesticide use would be at higher risk. We identified incident PD cases from French National Health Insurance databases (2010–2012). The proportion of land dedicated to 18 types of agricultural activities was defined at the canton of residence level. We examined the association between agricultural activities and PD age/sex-standardized incidence ratios using multivariable multilevel Poisson regression adjusted for smoking, deprivation index, density of neurologists, and rurality (proportion of agricultural land); we used a false discovery rate approach to correct for multiple comparisons and compute q-values. We also compared incidence in clusters of cantons with similar agricultural characteristics (k-means algorithm). We identified 69,010 incident PD cases. Rurality was associated with higher PD incidence (p < 0.001). Cantons with higher density of vineyards displayed the strongest association (RR_{top/bottom quartile} = 1.102, 95% CI = 1.049–1.158; q-trend = 0.040). This association was similar in men, women, and non-farmers, stronger in older than younger persons, and present in all French regions. Persons living in the cluster with greatest vineyards density had 8.5% (4.4–12.6%) higher PD incidence (p < 0.001). In France, vineyards rank among the crops that require most intense pesticide use. Regions with greater presence of vineyards are characterized by higher PD risk; non-professional pesticides exposure is a possible explanation.     

Patients’ and caregivers’ perspectives: assessing an intensive rehabilitation programme and outcomes in Huntington’s disease

Aim  

To investigate the subjective evaluation of an intensive rehabilitation programme and outcomes by people with Huntington’s disease (HD) and their caregivers.     

Non-pharmacologic management of sleep disturbance in Alzheimer’s disease

Sleep and wake in Alzheimer’s disease (AD) are often fragmented as manifested by bouts of wakefulness at night and napping during the day. Management of sleep disturbances in AD is important because of their negative impact on both patients and caregivers. Pharmacological treatments, mainly sedative-hypnotics and antipsychotics, are often used but can be associated with significant adverse effects. Non-pharmacological treatments represent a beneficial alternative approach to the management of sleep disturbances in AD since they are associated with fewer adverse effects and their efficacy can be sustained after treatment has been completed. The aim of this article is to review non-pharmacological treatments, such as sleep hygiene, sleep restriction therapy (SRT), cognitive behavioral therapy (CBT), light therapy, and continuous positive airway pressure (CPAP), for the management of sleep/wake disturbances in AD.