Survival Patterns of Human Prion Diseases in Spain, 1998-2018: Clinical Phenotypes and Etiological Clues.

Background: Human transmissible spongiform encephalopathies (TSEs) are a group of fatal neurodegenerative disorders of short duration. There are few studies on TSE survival. This study sought to analyze the survival and related factors of a TSE patient cohort, based on a nationwide surveillance system in Spain.

Association of disability with mortality in the Spanish adult non-institutionalised population.

Background: There are scant studies focused on measuring the association between disability and all-cause mortality based on large representative national samples of the community-dwelling adult population; moreover, the number of such studies which also include cause-specific mortality is yet lower.

Methods: Longitudinal cohort study that used baseline data from 162 381 adults who participated in a countrywide disability survey (2008). A nationally representative sample was selected and interviewed in their homes.

Human prion disease surveillance in Spain, 1993-2018: an overview.

In Spain, human transmissible spongiform encephalopathies (TSEs) have been undergoing continuous surveillance for over 25 years. In 1995, the system was launched as an EU Concerted Action, with EU surveillance network procedures being incorporated from 2002 onwards. The aim of this report was to describe performance and outcomes of this surveillance system across the period 1993-2018.

Validity of the Global Activity Limitation Indicator (GALI) to evaluate severity of disability.

Our aim was to estimate the validity of the Global Activity Limitation Indicator (GALI) when measuring the severity level of disability. Data came from 153 residents of Spain, who requested an evaluation of their degree of disability. We compared disability classifications (severe vs.

Age at onset in genetic prion disease and the design of preventive clinical trials.

Objective: To determine whether preventive trials in genetic prion disease could be designed to follow presymptomatic mutation carriers to onset of disease.

Methods: We assembled age at onset or death data from 1,094 individuals with high penetrance mutations in the prion protein gene () in order to generate survival and hazard curves and test for genetic modifiers of age at onset. We used formulae and simulations to estimate statistical power for clinical trials.

Facility ownership and mortality among older adults residing in care homes.

Background And Objectives: Nursing or care home characteristics may have a long-term impact on the residents' mortality risks that has not been studied previously. The study's main objective was to assess the association between facility ownership and long-term, all-cause mortality.

Research Design And Methods: We conducted a mortality follow-up study on a cohort of 611 nursing-home residents in the city Madrid, Spain, from their 1998-1999 baseline interviews up to September 2013.

Risk of transmission of sporadic Creutzfeldt-Jakob disease by surgical procedures: systematic reviews and quality of evidence.

Background: Sporadic Creutzfeldt-Jakob disease (sCJD) is potentially transmissible to humans.

Objective: This study aimed to summarise and rate the quality of the evidence of the association between surgery and sCJD.

Design And Methods: Firstly, we conducted systematic reviews and meta-analyses of case-control studies with major surgical procedures as exposures under study.

Parkinson's Disease Subtypes Identified from Cluster Analysis of Motor and Non-motor Symptoms.

Parkinson's disease is now considered a complex, multi-peptide, central, and peripheral nervous system disorder with considerable clinical heterogeneity. Non-motor symptoms play a key role in the trajectory of Parkinson's disease, from prodromal premotor to end stages. To understand the clinical heterogeneity of Parkinson's disease, this study used cluster analysis to search for subtypes from a large, multi-center, international, and well-characterized cohort of Parkinson's disease patients across all motor stages, using a combination of cardinal motor features (bradykinesia, rigidity, tremor, axial signs) and, for the first time, specific validated rater-based non-motor symptom scales.

Similarities of Variant Creutzfeldt-Jakob Disease Strain in Mother and Son in Spain to UK Reference Case.

We investigated transmission characteristics of variant Creutzfeldt-Jakob disease in a mother and son from Spain. Despite differences in patient age and disease manifestations, we found the same strain properties in these patients as in UK vCJD cases. A single strain of agent appears to be responsible for all vCJD cases to date.

Spinal Cord Injury due to Tumour or Metastasis in Aragón, Northeastern Spain (1991-2008): Incidence, Time Trends, and Neurological Function.

Purpose: Understanding the presentation of spinal cord injury (SCI) due to tumours considering population distribution and temporal trends is key to managing SCI health services. This study quantified incidence rates, function scores, and trends of SCI due to tumour or metastasis over an 18-year time period in a defined region in Spain.

Methods: A retrospective cohort study included in-and outpatients with nontraumatic SCI due to tumour or metastasis admitted to a metropolitan hospital in Spain between 1991 and 2008.

A protective personal factor against disability and dependence in the elderly: an ordinal regression analysis with nine geographically-defined samples from Spain.

Background: Sense of Coherence (SOC) is defined as a tendency to perceive life experiences as comprehensible, manageable and meaningful. The construct is split in three major domains: Comprehensibility, Manageability, and Meaningfulness. SOC has been associated with successful coping strategies in the face of illness and traumatic events and is a predictor of self-reported and objective health in a variety of contexts.

Urinary incontinence and mortality among older adults residing in care homes.

Aim: To assess the association between baseline urinary incontinence and long-term, all-cause mortality.

Background: Urinary incontinence is a common disorder among older institutionalised adults, with important consequences for morbidity and quality of life. Moreover, while it is a consistent mortality marker, the extent to which this association might be causal remains controversial.

Prevalence of and factors associated with homebound status among adults in urban and rural Spanish populations.

Background: There is a marked growth in the number of homebound older adults, due mainly to increased life expectancy. Although this group has special characteristics and needs, it has not been properly studied. This study thus aimed to measure the prevalence of homebound status in a community-dwelling population, and its association with both socio-demographic, medical and functional characteristics and the use of health care and social services.

Etiologic Framework for the Study of Neurodegenerative Disorders as Well as Vascular and Metabolic Comorbidities on the Grounds of Shared Epidemiologic and Biologic Features.

Background: During the last two decades, protein aggregation at all organismal levels, from viruses to humans, has emerged from a neglected area of protein science to become a central issue in biology and biomedicine. This article constitutes a risk-based review aimed at supporting an etiologic scenario of selected, sporadic, protein-associated, i.e.

Associations between chronic conditions, body functions, activity limitations and participation restrictions: a cross-sectional approach in Spanish non-clinical populations.

Objectives: To analyse the relationships between chronic conditions, body functions, activity limitations and participation restrictions in the International Classification of Functioning, Disability and Health (ICF) framework.

Design: A cross-sectional study.

Setting: 2 geographical areas in the Autonomous Region of Aragon, Spain, namely, a rural area, Cinco Villas, and an urban area in the city of Zaragoza.

Guillain-Barré syndrome following the 2009 pandemic monovalent and seasonal trivalent influenza vaccination campaigns in Spain from 2009 to 2011: outcomes from active surveillance by a neurologist network, and records from a country-wide hospital discharge database.

Background: Studies have shown a slight excess risk in Guillain-Barré syndrome (GBS) incidence associated with A(H1N1)pdm09 vaccination campaign and seasonal trivalent influenza vaccine immunisations in 2009-2010. We aimed to assess the incidence of GBS as a potential adverse effect of A(H1N1)pdm09 vaccination.

Methods: A neurologist-led network, active at the neurology departments of ten general hospitals serving an adult population of 4.

Drivers: A Biologically Contextualized, Cross-Inferential View of the Epidemiology of Neurodegenerative Disorders.

Background: Sutherland et al. (2011) suggested that, instead of risk factors for single neurodegenerative disorders (NDDs), there was a need to identify specific "drivers", i.e.

Quantifying prion disease penetrance using large population control cohorts.

More than 100,000 genetic variants are reported to cause Mendelian disease in humans, but the penetrance-the probability that a carrier of the purported disease-causing genotype will indeed develop the disease-is generally unknown. We assess the impact of variants in the prion protein gene (PRNP) on the risk of prion disease by analyzing 16,025 prion disease cases, 60,706 population control exomes, and 531,575 individuals genotyped by 23andMe Inc. We show that missense variants in PRNP previously reported to be pathogenic are at least 30 times more common in the population than expected on the basis of genetic prion disease prevalence.

Parkinson Symptoms and Health Related Quality of Life as Predictors of Costs: A Longitudinal Observational Study with Linear Mixed Model Analysis.

Objective: To estimate the magnitude in which Parkinson's disease (PD) symptoms and health- related quality of life (HRQoL) determined PD costs over a 4-year period.

Materials And Methods: Data collected during 3-month, each year, for 4 years, from the ELEP study, included sociodemographic, clinical and use of resources information. Costs were calculated yearly, as mean 3-month costs/patient and updated to Spanish €, 2012.

Accuracy of screening instruments for detection of neuropsychiatric syndromes in Parkinson's disease.

Parkinson's disease includes neuropsychiatric manifestations, such as depression, anxiety, apathy, psychosis, and impulse control disorders, which often are unreported by patients and caregivers or undetected by doctors. Given their substantial impact on patients and caregivers as well as the existence of effective therapies for some of these disorders, screening for neuropsychiatric symptoms is important. Instruments for screening have a particular methodology for validation, and their performance is expressed in terms of accuracy compared with formal diagnostic criteria.

Comparative Incidence of Conformational, Neurodegenerative Disorders.

Background: The purpose of this study was to identify incidence and survival patterns in conformational neurodegenerative disorders (CNDDs).

Methods: We identified 2563 reports on the incidence of eight conditions representing sporadic, acquired and genetic, protein-associated, i.e.

Discordance between physician-rated health and an objective health measure among institutionalized older people.

Background: Although physician-rated health is emerging as a potentially useful variable in research, with implications in practice, it has not been analyzed. Moreover, one of its most important aspects, namely, concordance with patients' objective health state, has not been investigated. This study sought to measure concordance between physician-rated health and an objective health measure, and assess both measures' validity in predicting death.