Incidence of multiple sclerosis among European Economic Area populations, 1985-2009: the framework for monitoring

Background: A debate surrounding multiple sclerosis epidemiology has centred on time-related incidence increases and the need of monitoring. The purpose of this study is to reassess multiple sclerosis incidence in the European Economic Area. Methods: We conducted a systematic review of literature from 1965 onwards and integrated elements of original research, including requested or completed data by surveys authors and specific analyses. Results: The review of 5323 documents yielded ten studies for age- and sex-specific analyses, and 21 studies for time-trend analysis of single data sets. After 1985, the incidence of multiple sclerosis ranged from 1.12 to 6.96 per 100,000 population, was higher in females, tripled with latitude, and doubled with study midpoint year. The north registered increasing trends from the 1960s and 1970s, with a historic drop in the Faroe Islands, and fairly stable data in the period 1980-2000; incidence rose in Italian and French populations in the period 1970-2000, in Evros (Greece) in the 1980s, and in the French West Indies in around 2000. Conclusions: We conclude that the increase in multiple sclerosis incidence is only apparent, and that it is not specific to women. Monitoring of multiple sclerosis incidence might be appropriate for the European Economic AreaS

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. Methods: Survival analyses were performed on 1,530 cases diagnosed across the period 1998–2018 in Spain. We calculated median survival times and plotted survival curves using the Kaplan–Meier method for all cases and for sporadic TSE (sTSE) and genetic TSE (gTSE). Crude and adjusted Cox proportional hazard models were used to identify variables associated with shorter survival. Findings: Median age at onset decreased from the sporadic forms to gTSE and, lastly, to acquired TSE. Overall median and interquartile range (IQR) survival time was 5.2 (IQR, 3.0–11.7) months and 4.9 (IQR, 2.8–10.8) months in sporadic cases and 9 (IQR, 4.9 to over 12) months in genetic cases, p < 0.001. Male sex, older age at onset, presence of 14-3-3 protein, typical MRI, and MM and VV polymorphisms at codon 129 were associated with shorter survival. gTSE showed higher survival in crude comparisons but not after adjustment. Interpretation: TSE survival in Spain replicates both the magnitude of that shown and the TSE entity-specific population patterns observed in Western countries but differs from features described in Asian populations, such as the Japanese. The reduction in differences in survival between gTSE and sTSE on adjusting for covariates and international patterns might support the view that gTSE and sTSE share causal and pathophysiological features.This work was basically funded by the Spain’s Ministry of Health and the Carlos III Health Institute (research grant no. PI08/0139) and Consortium for Biomedical Research in Neurodegenerative Diseases (CIBERNED) networks (QLRG3-CT-2002-81223). Role of funders: The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.S

Discapacidad y mortalidad en la encuesta nacional de discapacidad de España

Comunicación presentada en las II Jornada del Centro Nacional de Epidemiología - 2021.El objetivo del proyecto es medir la asociación entre discapacidad y mortalidad, general y por causas, en la población española adulta no institucionalizada. Las conclusiones a las que se han llegado son: 1. La mortalidad por todas las causas es claramente mayor en los adultos con discapacidad. 2. Se necesitan medidas preventivas e iniciativas de promoción de la salud para reducir el riesgo de mortalidad en la población con discapacidad. 3. Se debe prestar especial atención a las personas discapacitadas con algunas enfermedades específicas

Discapacidad, dependencia, apoyo y servicios en poblaciones españolas, 2008-2009

La presentación da una explica los modelos de la discapacidad y las distintas clasificaciones existentes. Los objetivos del estudio son 3: objetivo 1. Valorar datos en perspectiva CIF, prevalencia de discapacidad global y en dominios, con puntuación individual y Describir patrones de gravedad; objetivo 2: Describir la prevalencia de discapacidad en una población >50 años, de un área rural-semirrural de España y objetivo 3: Describir relaciones entre discapacidad y servicios asistenciales de larga duración (SALD) y el impacto de la Ley de autonomía personal de 2006.N

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. Neurology and public health specialists from every region reported cases to a central hub at the Carlos III Health Institute, Madrid. In all, eight accidentally transmitted cases and five definite variant Creutzfeldt-Jakob disease (vCJD) patients were reported. All vCJD cases were diagnosed between 2005 and 2008. Two of these were family/dietary-related and spatially linked to a third. Yearly incidence of sporadic CJD per million was 1.25 across the period 1998-2018, and displayed a north-south gradient with the highest incidence in La Rioja, Navarre and the Basque Country. Genetic TSEs were observed to be clustered in the Basque Country, with a 4-fold incidence over the national rate. A total of 120 (5.6%) non-TSE sporadic, conformational, rapidly progressing neurodegenerative and vascular brain disorders were reported as suspect CJD. We conclude that TSEs in Spain displayed geographically uneven, stable medium incidences for the sporadic and genetic forms, a temporal and spatial family cluster for vCJD, and decreasing numbers for dura-mater-associated forms. The vCJD surveillance, framed within the EU network, might require continuing to cover all prion disorders. There is need for further strategic surveillance research focusing on case definition of rapid-course, conformational encephalopathies and surgical risk.This work was basically funded by the Spain’s Ministry of Health and the Carlos III Health Institute. Additional funding was obtained from the EU Research Commission, Concerted Actions (BIOMED2 Contract No. BMH4-CT97-2216, QLRG3-CT-2002-81223), EU Joint Program – Neurodegenerative Disease Research (JPND – DEMTEST, Spanish Health Research Fund, FIS PI11/03021 and PI12/00045), EUROCJD (funded by DG SANCO, 2003201), research grants by the Carlos III Health Institute (PI08/0139), NEUROPRION (an EU Network of Excellence funded by Framework 6 Programme, FOOD CT 2004 056579), the Spanish Centro de Investigaciones en Enfermedades Neurológicas (CIEN C03-06), and Consortium for Biomedical Research in Neurodegenerative Diseases (CIBERNED) networks.S

ICF-Based Disability Survey in a Rural Population of Adults and Older Adults Living in Cinco Villas, Northeastern Spain: Design, Methods and Population Characteristics

Background: This article describes the methods of a door-to-door screening survey exploring the distribution of disability and its major determinants in northeastern Spain. This study will set the basis for the development of disability-related services for the rural elderly in northeastern Spain. Methods: The probabilistic sample was composed of 1,354 de facto residents from a population of 12,784 Social Security card holders (age: 6 50 years). Cognitive and disability screenings were conducted (period: June 2008-June 2009). Screening instruments were the MMSE and the World Health Organization Disability Assessment Schedule. Participants screened positive for disability underwent an assessment protocol focusing on primary care diagnoses, disability, lifestyle, and social and health service usage. Participants screened positive for cognitive functioning went through in-depth neurological evaluation. Results: The study sample is described. Usable data were available for 1,216 participants. A total of 625 individuals (51.4%) scored within the positive range in the disability screening, while 135 (11.1%) scored within the positive range of the cognitive screening. The proportion of positively screened individuals was higher for women and increased with age. Conclusions: Screening surveys represent a feasible design for examining the distribution of disability and its determinants among the elderly. Data quality may benefit from methodological developments tailored to rural populations with a low education level. Copyright (C) 2010 S. Karger AG, Base

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. PARTICIPANTS: 864 individuals selected by simple random sampling from the register of Social Security card holders, aged 50 years and over, positive to disability screening. MAIN OUTCOME MEASURES: ICF Checklist-body function domains, WHO Disability Assessment Schedule 2.0 (WHODAS 2.0, 36-item (WHODAS-36)) global scores and medical diagnoses (chronic conditions) from primary care records. RESULTS: Mild disability (WHODAS-36 level 5-24%) was present in 51.5% of the sample. In the adjusted ordinal regression model with WHODAS-36 as the dependent variable, disability was substantially associated with moderate-to-complete impairment in the following functions: mental, OR 212.8 (95% CI 72 to 628.9); neuromusculoskeletal, OR 44.8 (24.2 to 82.8); and sensory and pain, OR 6.3 (3.5 to 11.2). In the relationship between health conditions and body function impairments, the strongest links were seen for: dementia with mental functions, OR 50.6 (25.1 to 102.1); cerebrovascular disease with neuromusculoskeletal function, OR 5.8 (3.5 to 9.7); and chronic renal failure with sensory function and pain, OR 3.0 (1.49 to 6.4). Dementia, OR 8.1 (4.4 to 14.7) and cerebrovascular disease, OR 4.1 (2.7 to 6.4) were associated with WHODAS-36 scores. CONCLUSIONS: Body functions are heterogeneously linked to limitations in activities and restrictions on participation, with the highest impact being due to mental and musculoskeletal functions. This may be relevant for disability assessment and intervention design, particularly if defined on a body function basis. Control of specific health conditions, such as dementia and cerebrovascular disease, appears to be paramount in reducing disability among persons aged 50 years and over.This study was funded by the Carlos III Institute of Health (EPI projects 1637/06 and 1530/07; Health Research Fund grants PI06/1098 and PI07/90206), Convenio Marco IMSERSO-ISCIII reference number STVI 1282/ 15, Consortium for Biomedical Research in Neurodegenerative Diseases (Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas, CIBERNED), Zaragoza Regional Authority and Farasdués Foundation.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-SträusslerScheinker (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.N

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-Barre 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 4.68 million, conducted GBS surveillance in Spain in 2009-2011. The network, established in 1996, carried out a retrospective and a prospective study to estimate monthly alarm thresholds in GBS incidence and tested them in 1998-1999 in a pilot study. Such incidence thresholds additionally to observation of GBS cases with immunisation antecedent in the 42 days prior to clinical onset were taken as alarm signals for 2009-2011, since November 2009 onwards. For purpose of surveillance, in 2009 we updated both the available centres and the populations served by the network. We also did a retrospective countrywide review of hospital-discharged patients having ICD-9-CM code 357.0 (acute infective polyneuritis) as their principal diagnosis from January 2009 to December 2011. Results: Among 141 confirmed of 148 notified cases of GBS or Miller-Fisher syndrome, Brighton 1-2 criteria in 96 %, not a single patient was identified with clinical onset during the 42-day time interval following A(H1N1) pdm09 vaccination. In contrast, seven cases were seen during a similar period after seasonal campaigns. Monthly incidence figures did not, however, exceed the upper 95 % CI limit of expected incidence. A retrospective countrywide review of the registry of hospital-discharged patients having ICD-9-CM code 357.0 (acute infective polyneuritis) as their principal diagnosis did not suggest higher admission rates in critical months across the period December 2009-February 2010. Conclusions: Despite limited power and underlying reporting bias in 2010-2011, an increase in GBS incidence over background GBS, associated with A(H1N1) pdm09 monovalent or trivalent influenza immunisations, appears unlikely