Spatial distribution of Parkinson's disease mortality in Spain, 1989-1998, as a guide for focused aetiological research or health-care intervention

<p>Abstract</p> <p>Background</p> <p>Aetiologically, genetic and environmental factors having an uneven spatial distribution may underlie Parkinson's disease (PD). Undiagnosis of PD in selected regions might have limited access to treatment with levodopa and simultaneously, if present at death, determined PD underreporting at the death record. The purpose of this study was to describe and analyse municipal mortality due to PD in Spain in aetiological and interventional perspective.</p> <p>Methods</p> <p>PD mortality at a municipal level was modelled using the Besag-York- Molliè autoregressive spatial model, combining demographic information with cause-of-death diagnostic data (International Classification of Diseases 9^thRevision (ICD-9) code 332.0). Municipal relative risks (RRs) were independently estimated for women, men and both sexes, and plotted on maps depicting smoothed RR estimates and the distribution of the posterior probability of RR>1.</p> <p>Results</p> <p>A south-north gradient, with large geographical areas suggesting clustered towns with high mortality, was seen in Asturias, the Basque Country, Balearic Islands and, particularly, in the Lower Ebro valley around Tarragona. Similarly, there was a suggestion that lowest mortality was clustered in the south-east and south-west. We identified some isolated or clustered municipalities with high mortality that were situated near industrial plants reported to be associated with environmental xenobiotic emissions. However, the same pattern was also observed for some cities with low mortality.</p> <p>Conclusion</p> <p>Municipal PD mortality in Spain was unevenly distributed. Patterns were roughly similar to reported provincial PD mortality and use of levodopa. While the overall pattern appears to result from spatially selective PD undiagnosis, and can not be ascribed to industrial emissions, it can not be excluded that selected "hot spots" reflect genetic factors and/or environmental exposures inducing parkinsonism. A few municipal populations, located in low-mortality-risk areas in the vicinity of polluting plants or registering high excess PD mortality, might constitute a priority for conducting direct etiological studies. Additionally, interventions aimed to reduce potential PD undiagnosis might be most appropriate in the South.</p

Tissue classification for the epidemiological assessment of surgical transmission of sporadic Creutzfeldt-Jakob disease. A proposal on hypothetical risk levels

BACKGROUND: Epidemiological studies on the potential role of surgery in Creutzfeldt-Jakob Disease transmission have disclosed associations with history of specific surgical interventions or reported negative results. METHODS: Within the context of a case-control study designed to address surgical risk of sporadic Creutzfeldt-Jakob Disease in Nordic European countries (EUROSURGYCJD Project), a strategy was adopted to categorise reported surgical procedures in terms of potential risk of Creutzfeldt-Jakob Disease acquisition. We took into account elements of biological plausibility, either clinically or experimentally demonstrated, such as tissue infectivity, PrP expression content or successful route of infection. RESULTS: We propose a classification of exposed tissues and anatomic structures, drawn up on the basis of their specific putative role as entry site for prion transmission through contact with surgical instruments that are not fully decontaminated. CONCLUSIONS: This classification can serve as a reference, both in our study and in further epidemiological research, for categorisation of surgical procedures in terms of risk level of Creutzfeldt-Jakob Disease acquisition

Validación de escalas de medida de discapacidad según la CIF en aragoneses de edad media y avanzada

Introducción La investigación sobre el funcionamiento se ha vuelto cada vez más necesaria para describir la salud de las personas. En investigación epidemiológica contamos con pocos estudios e instrumentos para la adecuada medida de la discapacidad dada la complejidad de su definición y de las interacciones entre la persona y su entorno. La discapacidad en las personas mayores es altamente prevalente en España, donde el aumento de la longevidad va a suponer un incremento de la prevalencia de las enfermedades crónicas. La OMS, desde una mirada psicosocial aprobó en 2001 la Clasificación Internacional del Funcionamiento, de la Discapacidad y de la Salud (CIF), desarrollando dos herramientas para medir el funcionamiento en la evaluación de la salud: el World Health Organization Disability Assessment Schedule 2.0 (WHODAS 2.0) para uso epidemiológico y la Lista de Comprobación CIF (LCCIF) para uso clínico.Objetivos 1. Describir las frecuencias de discapacidad en diversos grupos de población de edad media y avanzada de la Comunidad Autónoma de Aragón utilizando los instrumentos de medida recomendados por la Clasificación CIF de la OMS. 2. Determinar la sensibilidad y especificidad de los instrumentos WHODAS 12 y WHODAS 36 para el cribado y medida de la discapacidad con respecto de la LCCIF como estándar. 3. Cuantificar los porcentajes de limitaciones en la Actividad y restricciones en la Participación de la LCCIF con los calificadores de capacidad y desempeño. 4. Analizar la concordancia entre ambos calificadores de capacidad y desempeño y la magnitud de sus diferencias, con objeto de diseñar estudios que identifiquen factores de riesgo, barreras y facilitadores ambientales de discapacidad en esta población.Metodología Se realizó un estudio transversal entre los años 2008-2011, utilizando los datos extraídos de una encuesta poblacional en dos áreas geográficas (una rural y otra urbana) de la Comunidad Autónoma de Aragón en el Noreste de España. Los participantes fueron personas ≥50 años. Para detectar los determinantes de discapacidad más relevantes, los participantes fueron doblemente cribados por discapacidad y deterioro cognitivo. El cribado de discapacidad se realizó mediante un instrumento de metodología CIF, el WHODAS de 12 preguntas y el de deterioro cognitivo con el Examen Cognoscitivo Mini-Mental (MEC). Los participantes con cribado positivo y todos los de una muestra probabilística como población de validación, se sometieron a un protocolo de evaluación, donde se estudiaba en detalle su discapacidad en visita puerta a puerta mediante el WHODAS de 36 preguntas, la LCCIF, estado de salud y una encuesta estructurada relativa a apoyo y servicios. Se midió la sensibilidad y la especificidad del WHODAS de 12 y 36 preguntas con respecto de la LCCIF como estándar. Se analizaron las diferencias y la concordancia entre los calificadores de capacidad y desempeño para los dominios del componente de Actividades y participación de la LCCIF.Resultados El sexo femenino, la edad avanzada y el nivel de estudios bajo están sobre-representados en la población con discapacidad moderada y grave. Tomando como estándar para detectar discapacidad la LCCIF, los valores de sensibilidad, los valores de VP- y de 1/RP(-) son algo más altos en el WHODAS 36 que el 12. La especificidad, VP+ y RP(+) toman valores similares en ambas versiones de WHODAS. Los dominios con peor capacidad y peor desempeño para discapacidad grave/total fueron: Tareas y demandas generales, Vida Doméstica y Movilidad. Los dominios con concordancia más baja entre capacidad y desempeño fueron: Vida Doméstica y Autocuidado.Conclusiones El WHODAS 2.0 es una herramienta válida y útil para para detectar a personas con discapacidad. Los resultados apuntan a posibles discordancias entre las medidas de puntuación de la LCCIF y el WHODAS 2.0. La concordancia entre capacidad y desempeño en los cuatro niveles de puntuación fue alta. Este estudio constituye un primer paso basado en la CIF hacia un abordaje más completo de la discapacidad.<br /

Encefalopatías espongiformes transmisibles humanas en España.

La perspectiva poblacional de la enfermedad de Creutzfeldt-Jakob (ECJ) y otras encefalopat&iacute;as espongiformes transmisibles en Espa&ntilde;a es razonablemente conocida desde 1995. Durante estos 15 a&ntilde;os se asiste probablemente a la desaparici&oacute;n de las formas iatrog&eacute;nicas por implantes de duramadre y al tard&iacute;o surgimiento y cese de un reducido brote de la variante de la ECJ. Persisten las frecuencias medias de ECJ espor&aacute;dica, escasamente diferenciables geogr&aacute;ficamente a excepci&oacute;n de las m&aacute;s altas en el Pa&iacute;s Vasco donde se da tambi&eacute;n la &uacute;nica, elevada incidencia de formas gen&eacute;ticas. Es la ECJ espor&aacute;dica la que viene a constituirmundialmente un nuevo reto, en el momento en el que la atenci&oacute;n en salud p&uacute;blica se comienza a dirigir hacia su posible transmisi&oacute;n nosocomial

Prevalence of Parkinson's disease in Cantalejo, Spain: A door-to-door survey

We assessed the prevalence of Parkinson’s disease in Cantalejo, Spain. In 1994, we screened 1,579 persons (age $ 40 years) using a high-sensitivity method. Cases fulfilling established clinical criteria were followed for a minimum of 3 years. Prevalences were compared with those from other doorto- door surveys. We detected 27 individuals with parkinsonism, 20 of whom had Parkinson’s disease. The prevalence of Parkinson’s disease increased with age and, when age-adjusted to European standards, was 9.01 per 1,000 (age 40 years and over; 10.78 in men and 5.23 in women). Of the 11 men, three were in Hoehn & Yahr grades III–IV, but six of the nine women were more severely affected. Overall, we found 18 newly diagnosed cases of parkinsonism, 13 of which were Parkinson’s disease, and the majority of which were in men aged 80 years or older with a mean duration of illness of 5 years. Our prevalence figures are the highest reported, apparently because of the inclusion of several very elderly men. Parkinson’s disease in Cantalejo is less severe in men than in women, particularly in those newly diagnosed. Despite the low numbers, the high prevalence and sex-related pattern are unexplained but they probably relate to the high sensitivity of the screening method. © 2002 Movement Disorder Societ

Nosocomial transmission of sporadic Creutzfeldt–Jakob disease: results from a risk-based assessment of surgical interventions

OBJECTIVES: Evidence of surgical transmission of sporadic Creutzfeldt-Jakob disease (sCJD) remains debatable in part due to misclassification of exposure levels. In a registry-based case-control study, the authors applied a risk-based classification of surgical interventions to determine the association between a history of surgery and sCJD. DESIGN: Case-control study, allowing for detailed analysis according to time since exposure. SETTING: National populations of Denmark and Sweden. PARTICIPANTS: From national registries of Denmark and Sweden, the authors included 167 definite and probable sCJD cases with onset during the period 1987-2003, 835 age-, sex- and residence-matched controls and 2224 unmatched. Surgical procedures were categorised by anatomical structure and presumed risk of transmission level. The authors used logistic regression to determine the odds ratio (OR) for sCJD by surgical interventions in specified time-windows before disease-onset. RESULTS: From comparisons with matched controls, procedures involving retina and optic nerve were associated with an increased risk at a latency of ≥1 year OR (95% CI) 5.53 (1.08 to 28.0). At latencies of 10 to 19 years, interventions on peripheral nerves 4.41 (1.17 to 16.6) and skeletal muscle 1.58 (1.01 to 2.48) were directly associated. Interventions on blood vessels 4.54 (1.01 to 20.0), peritoneum 2.38 (1.14 to 4.96) and skeletal muscle 2.04 (1.06 to 3.92), interventions conducted by vaginal approach 2.26 (1.14 to 4.47) and a pooled category of lower-risk procedures 2.81 (1.62 to 4.88) had an increased risk after ≥20 years. Similar results were found when comparing with unmatched controls. INTERPRETATION: This observation is in concordance with animal models of prion neuroinvasion and is likely to represent a causal relation of surgery with a non-negligible proportion of sCJD cases.Funding was obtained from The Research Commission EU, Concerted Action QLRG3-CT-2002-81223, NEUROPRION, and the Spanish RECSP C03-09, CIEN C03-06 and CIBERNED networks.S

Medical, environmental and personal factors of disability in the elderly in Spain: a screening survey based on the International Classification of Functioning

Malalties cròniques; Demència; Avaluació de la discapacitatEnfermedades crónicas; Demencia; Evaluación de la discapacidadChronic diseases; Dementia; Disability evaluationObjectives: The International Classification of Functioning, Disability and Health (ICF) advocates a multifactorial and multifaceted conceptualization of disability. The objective of this study was to ascertain major medical, environmental and personal determinants of severe/extreme disability among the elderly population in Spain. The assessment scheme was consistent with the ICF model of disability.Methods: Nine populations contributed probabilistic or geographically-defined samples following a two-phase screening design. The Mini-Mental State Examination and the 12-item version of the World Health Organization-Disability Assessment Schedule, 2(nd) ed. (WHO-DAS II), were used as cognitive and disability screening tools, respectively. Positively screened individuals underwent clinical work-up for dementia and were administered the 36-item version of the WHO-DAS II to estimate ICF disability levels. We used logistic regression for the purposes of data combination, adjusted for age and sex in all analyses. Results: The sample was composed of 503 participants aged ≥ 75 years. Alzheimeŕs disease and depression were highly predictive of severe/extreme disability (OR: 17.40, 3.71). Good access to social services was strongly associated with a low level or absence of disability (OR: 0.05 to 0.18). Very difficult access to services and having dementia or another psychiatric disorder were associated with an increase in disability (OR: 66.06). There was also a significant interaction effect between access to services and neurological disorders (OR: 12.74).Conclusions: Disability is highly prevalent among the Spanish elderly and is influenced by medical, social and personal factors. Disability could potentially be reduced by ensuring access to social services, preventing dementia and stroke, and treating depression.This project, led by J. de Pedro-Cuesta, was supported by thePfizer Foundation and by the RECSP C03-09, CIEN C03-06 and CIBERNED and CIBERSAM research network

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. Mixed linear models were performed to analyze total, direct and indirect costs based on symptoms and HRQoL. RESULTS: One-hundred and seventy four patients were included. Mean (SD) age: 63 (11) years, mean (SD) disease duration: 8 (6) years. Ninety-three percent were HY I, II or III (mild or moderate disease). Forty-nine percent remained in the same stage during the study period. Clinical evaluation and HRQoL scales showed relatively slight changes over time, demonstrating a stable group overall. Mean (SD) PD total costs augmented 92.5%, from € 2,082.17 (€ 2,889.86) in year 1 to € 4,008.6 (€ 7,757.35) in year 4. Total, direct and indirect cost incremented 45.96%, 35.63%, and 69.69% for mild disease, respectively, whereas increased 166.52% for total, 55.68% for direct and 347.85% for indirect cost in patients with moderate PD. For severe patients, cost remained almost the same throughout the study. For each additional point in the SCOPA-Motor scale total costs increased € 75.72 (p = 0.0174); for each additional point on SCOPA-Motor and the SCOPA-COG, direct costs incremented € 49.21 (p = 0.0094) and € 44.81 (p = 0.0404), respectively; and for each extra point on the pain scale, indirect costs increased € 16.31 (p = 0.0228). CONCLUSIONS: PD is an expensive disease in Spain. Disease progression and severity as well as motor and cognitive dysfunctions are major drivers of costs increments. Therapeutic measures aimed at controlling progression and symptoms could help contain disease expenses.Abbvie Pharmaceuticals has solely funded the publication of this study; however, the funder had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.S

Situación epidemiológica de las Encefalopatías Espongiformes Transmisibles Humanas en España

[ES] Las Encefalopatías Espongiformes Transmisibles Humanas (EETH) son enfermedades de declaración obligatoria, de baja incidencia, provocadas por depósitos de proteína priónica que cursan en general con demencia rápidamente progresiva. El Registro Nacional de EETH (RNEETH) recoge datos de los casos españoles desde 1995. En este informe se presentan las características de los casos de EETH de España. Los datos proceden de las notificaciones hechas por las unidades de vigilancia epidemiológica de las comunidades autónomas al RNEETH hasta el 1 de julio de 2016. La forma más común es la Enfermedad de Creutzfeldt-Jakob esporádica (ECJe, se desconoce la causa) cuya incidencia anual en España es de 1,1 casos por millón de habitantes y año. El grupo de edad con mayores tasas de ECJe es el de 70 a 79 años. El 55% de todos los casos son mujeres. En los años 2005, 2007 y 2008 se recogen cinco casos de variante de ECJ (vECJ), dos de ellos en una mujer y su hijo, única agrupación familiar descrita. En el RNEETH constan también siete casos de ECJ transmitida accidentalmente por implantes de duramadre y 152 casos genéticos: 68 en forma de Insomnio Familiar Letal (IFL), 81 de ECJ familiar (ECJf) y 3 de síndrome de Gerstmann-Sträussler-Scheinker (SGSS). El País Vasco presenta una incidencia de formas familiares por encima de las demás comunidades autónomas. Los datos que constan en el Registro Nacional de EETH reflejan una situación epidemiológica similar a la descrita en otros países de nuestro entorno salvo para las formas genéticas. [EN] Human Transmissible Spongiform Encephalopathies (HTSE) are notifiable diseases with lowincidence caused by prion protein (PrP) deposits. HTSE are generally characterized by rapidly progressive dementia. The National Registry of HTSE (NRHTSE) collects data from Spanish cases since 1995. This report summarizes the characteristics of the cases of HTSE in Spain. Data come from notifications made by the Epidemiological Surveillance Units of the Autonomous Regions to the NRHTSE until 1 July 2016. The most common form is sporadic Creutzfeldt-Jakob Disease (sCJD) whose annual incidence in Spain is 1.1 cases per million inhabitants per year. The age group with the highest CJD rates is 70 to 79 years. 55% are women. In 2005, 2007 and 2008, 5 cases of variant CJD (vCJD) were recorded, two of them in a woman and her son, the only family group described. In the NRHTSE there are 7 cases of CJD accidentally transmitted by dura mater implants and 152 genetic cases: 68 of Familial Insomnia (FFI), 81 of familial CJD (fCJD) and 3 of Gerstmann-Straüssler-Scheinker syndrome (GSSS). The Basque country has an incidence of family forms above the other Autonomous Regions. The data of the NRHTSE reflect an epidemiological situation similar to other countries of our environment except for genetic forms

Towards an age-dependent transmission model of acquired and sporadic Creutzfeldt-Jakob disease

INTRODUCTION: Sporadic Creutzfeldt-Jakob disease (sCJD) might be transmitted by surgery. The purpose of this study was to investigate potential susceptibility to sCJD from surgery at juvenile age and in early adulthood. METHODS: From Danish and Swedish national registries we identified 167 definite and probable sCJD cases with onset from 1987 through 2003, and 835 age-, sex- and residence-matched controls along with their surgical histories. Main, anatomically or etiologically classified surgical procedures followed by a ≥20-year lag were analyzed using logistic regression, and stratified by age at first-registered surgical discharge. RESULTS: The risk of having a diagnosis of CJD depended strongly on age at first surgery with odds ratio (OR) of 12.80 (95% CI 2.56-64.0) in patients <30 years, 3.04 (95% 1.26-7.33) in 30-39 years, and 1.75 (95% CI 0.89-3.45) in ≥40 years, for anatomically classified surgical procedures. Similar figures were obtained for etiologically classified surgical procedures. CONCLUSIONS: Risk of surgical-acquired sCJD depends on age at exposure; this pattern is similar to age-specific profiles reported for CJD accidentally transmitted by human pituitary-derived growth hormone and susceptibility curves for variant CJD estimated after adjustment for dietary exposure to bovine spongiform encephalopathy. There might be an age-at-exposure-related susceptibility to acquire all CJD forms, including sCJD from routine surgery.Funding was obtained from the EU Research Commission, Concerted Action QLRG3-CT-2002-81223 NEUROPRION and the Spanish Centro de Investigaciones en enfermedades Neurológicas (CIEN C03-06), and Consortium for Biomedical Research in Neurodegenerative Diseases (CIBERNED) networks, and from the Karolinska and Carlos III National Health Institutes. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.S