Incidence and prevalence of multiple sclerosis in Spain: a systematic review.

INTRODUCTION: Greater understanding of the prevalence and incidence of multiple sclerosis in Spain and their temporal trends is necessary to improve the allocation of healthcare resources and to study aetiological factors. METHODS: We performed a systematic search of the MedLine database and reviewed the reference lists of the articles gathered. We collected studies reporting prevalence or incidence rates of multiple sclerosis in any geographical location in Spain, with no time limits. In 70% of cases, data were extracted by 2 researchers (FGL and EAC); any discrepancies were resolved by consensus. RESULTS: We identified 51 prevalence and 33 incidence studies published between 1968 and 2018. In the adjusted analysis, the number of prevalent cases per 100 000 population increased by 26.6 (95% confidence interval [CI], 21.5-31.8) every 10 years. After adjusting for year and latitude, the number of incident cases per 100 000 population increased by 1.34 (95% CI, 0.98-1.69) every 10 years. We observed a trend toward higher prevalence and incidence rates at higher latitudes. CONCLUSIONS: The prevalence of multiple sclerosis in Spain has increased in recent decades, although case ascertainment appears to be incomplete in many studies. Incidence rates have also increased, but this may be due to recent improvements in the detection of new cases.

Disability, support and long-term social care of an elderly Spanish population, 2008-2009: an epidemiologic analysis.

BACKGROUND: Though poorly known, relationships between disability, need of help (dependency) and use of social services are crucial aspects of public health. The objective of this study was to describe the links between disability, officially assessed dependency, and social service use by an industrial population, and identify areas of inequity. METHODS: We took advantage of a door-to-door survey conducted in the Cinco Villas district, Spain, in 2008-2009, which provided data on disability, morbidity, and service use among 1216 residents aged ≥50 years, and officially assessed dependency under the 2006 Dependency Act (OAD). Using logistic regression, we combined data collected at homes/residences on 625 disability screened-positive participants, and administrative information on degree of OAD and benefits at date of visit. RESULTS: Based on 163 disabled persons, the prevalence of residential/community-care users was 13.4% overall, with 6.0% being market-provided, 2.5% supported by the 2006 Act, and 4.9% supported by other public funds. Of 111 OAD applicants, 30 had been assigned an OAD degree; in 29 cases this was the highest OAD degree, with 12 receiving direct support for residential care and 17 receiving home care. Compared to unassessed dependency, the highest OAD degree was linked to residential care (OR and 95% CI) 12.13 (3.86-38.16), declared non-professional care 10.99 (1.28-94.53), and publicly-funded, non-professional care 26.30 (3.36-205.88). In contrast, 43 persons, 58% of the severely/extremely disabled, community-dwelling sample population, 81% of whom were homebound, including 10 persons with OAD but no implemented service plan, made no use of any service, and of these, 40% lacked a non-professional carer. CONCLUSIONS: Formal service use in the Cinco Villas district attained ratios observed for established welfare systems but the publicly-funded proportion was lower. The 2006 Act had a modest, albeit significant, impact on support for non-professional carers and residential care, coexisting with a high prevalence of non-use of social services by severely disabled persons.

Association of detected depression and undetected depressive symptoms with long-term mortality in a cohort of institutionalised older people.

BACKGROUND: Studies on depression and mortality in nursing homes have shown inconclusive findings, and none has studied the role of detection. We sought to measure the association of depression with long-term all-cause mortality in institutionalised older people and evaluate a potential modification in the association by its detection status. METHODS: We selected a stratified cluster sample of 591 residents aged 75 years or older (mean age 84.5 years) living in residential and nursing homes of Madrid, Spain, who were free of severe cognitive impairment at the 1998-1999 baseline interview. Mortality was ascertained until age 105 years or September 2013 (median/maximum follow-up 4.8/15.2 years) through linkage to the Spanish National Death Index. Detected depression was defined at baseline as a physician's diagnosis or antidepressant use, undetected depression as significant depressive symptoms (score of 4 or higher on the ten-item version of the Geriatric Depression Scale) without documented diagnosis or treatment, and no depression as the absence of diagnosis, treatment, and symptoms. Constant and age-dependent hazard ratios for mortality comparing detected and undetected depression with no depression were estimated using Cox models, and absolute years of life gained and lost using Weibull models. RESULTS: The baseline prevalences of detected and undetected depression were 25.9 and 18.8%, respectively. A total of 499 participants died during 3575 person-years of follow-up. In models adjusted for age, sex, type of facility, number of chronic conditions, and functional dependency, overall depression was not associated with long-term all-cause mortality (hazard ratio 0.87, 95% confidence interval (CI): 0.70-1.08). However, compared with no depression, detected depression showed lower mortality (hazard ratio 0.63, 95% CI: 0.46-0.86), while undetected depression registered higher, not statistically significant, mortality (hazard ratio 1.35, 95% CI: 0.98-1.86). The median life expectancy increased by 1.8 years (95% CI: -3.1 to 6.7 years) in residents with detected depression and decreased by 6.3 years (95% CI: 2.6-10.1 years) in those undetected. Results were more marked in women than men and they were robust to the exclusion of antidepressants from the definition of depression and also to the use of a stricter cut-off for the presence of depressive symptoms. CONCLUSIONS: The long-term mortality risk associated with depression in nursing homes depends on its detection status, with better prognosis in residents with detected depression and worse in those undetected. The absolute impact of undetected depressive symptoms in terms of life expectancy can be prominent.

Analysis of disability using WHODAS 2.0 among the middle-aged and elderly in Cinco Villas, Spain.

BACKGROUND: The prevalence of disability, as defined by the International Classification of Functioning, Disability and Health (ICF), among the middle-aged and elderly population is poorly known. OBJECTIVE: To determine disability prevalence in a resident population sample aged ≥50 years, in the Cinco Villas district, Spain, from June 2008 through June 2009. METHODS: We used the WHODAS 2.0 36-item questionnaire to quantify the prevalence of disability, globally and by domain, together with a 13-item combined measure of three domains, Getting around, Self-care and Life activities, claimed to reflect the need of integrated services. In addition, we performed exploratory analyses of the relationship between disability and different variables using ordinal logistic regression. RESULTS: Disability was detected by global WHODAS score in 604 of a total of 1214 persons, i.e., a prevalence of 49.8% 95% CI (46.9-52.5), with the corresponding figures for mild, moderate, severe, and extreme disability being 26.8%, 16.0%, 7.6% and 0.1%, respectively. Disability increased with age, was higher among women, and for specific domains. Prevalence of severe/extreme disability among women vs. men was as follows: Getting around, 26.8% vs. 12.1%; Life activities, 25.2% vs. 6.8%; and Self-care, 9.5% vs. 6.0%. Disability was more frequent among subjects diagnosed with dementia, chronic liver disease, severe mental disease, and stroke. The abovementioned 13-item measure yielded prevalence figures for disability levels quite similar to those obtained using 36-item scores. CONCLUSIONS: For the first time, this study furnishes detailed disability prevalence figures and data on associated variables in a middle-aged and elderly Western population.

Capture-recapture as a potentially useful procedure for assessing prevalence of multiple sclerosis: methodologic exercise using Portuguese data.

BACKGROUND: Capture-recapture methods (CRMs) are well established in epidemiologic surveillance and considered useful for the task of correcting for case-finding limitations in multiple sclerosis (MS) prevalence surveys. To date, however, CRMs have been exclusively applied to crude prevalence figures. This study therefore sought to explore an age-specific application of this method to an urban Portuguese population of 229,342. METHODS: We used a CRM to correct for the age-specific prevalence of MS obtained from two data sources, i.e. general practitioners in three primary-care districts and a neurology unit at the referral hospital. The corrected figures were adjusted for age using the European standard population as reference. RESULTS: When applied to 95 MS patients, the CRM impact was highest at ages 50-59 years, with a 110% increase in cases where the corrected prevalence was highest, i.e. 181.8 (95% CI 75.7-287.9) per 100,000, and lowest, nil, at ages ≥70 years, with an unchanged corrected prevalence of 13.8. The crude prevalence of 41.4 per 100,000 increased by 36% to 56.20 per 100,000 when it was CRM- and age-adjusted. Source independence was poor. CONCLUSIONS: CRMs can be differentially applied to MS counts. Valid comparisons may require simultaneous adjustment for age and other variables, such as diagnostic delay and diagnostic criteria. CRM applications to crude figures and dependent sources should be approached with caution.

Health professions and risk of sporadic Creutzfeldt-Jakob disease, 1965 to 2010.

In 2009, a pathologist with sporadic Creutzfeldt-Jakob Disease (sCJD) was reported to the Spanish registry. This case prompted a request for information on health-related occupation in sCJD cases from countries participating in the European Creutzfeldt Jakob Disease Surveillance network (EuroCJD). Responses from registries in 21 countries revealed that of 8,321 registered cases, 65 physicians or dentists, two of whom were pathologists, and another 137 healthcare workers had been identified with sCJD. Five countries reported 15 physicians and 68 other health professionals among 2,968 controls or non-cases, suggesting no relative excess of sCJD among healthcare professionals. A literature review revealed: (i) 12 case or small case-series reports of 66 health professionals with sCJD, and (ii) five analytical studies on health-related occupation and sCJD, where statistically significant findings were solely observed for persons working at physicians' offices (odds ratio: 4.6 (95 CI: 1.2-17.6)). We conclude that a wide spectrum of medical specialities and health professions are represented in sCJD cases and that the data analysed do not support any overall increased occupational risk for health professionals. Nevertheless, there may be a specific risk in some professions associated with direct contact with high human-infectivity tissue.

Prevalence and European comparison of dementia in a ≥75-year-old composite population in Spain.

OBJECTIVES: To estimate dementia prevalence in Spain. MATERIALS AND METHODS: Nine probabilistic and geographically defined samples participated. A screening design based on the MMSE was implemented. Positively screened individuals underwent clinical evaluation. The total number of cases in Spain was estimated. Prevalence was confronted to that of other European countries. RESULTS: Five hundred and forty-six persons aged ≥75 participated, 49 had dementia (35 with Alzheimer's disease [AD], 10 with vascular dementia [VD], 4 other; 25 first diagnosed in the study). Age- and sex-adjusted prevalence and estimated nationwide cases were 7.5% (95% CI 5.4-9.7), 5.6 (95% CI 3.7-7.5) and 1.4 (95% CI 0.5-2.3), and 290,000 (95% CI 208,000-372,000), 214,000 (95% CI 141,000-288,000) and 54,000 (95% CI 20,000-88,000) for dementia, AD and VD, respectively. CONCLUSIONS: Dementia prevalence in Spain is comparable to other European populations, while a high number of undiagnosed cases live in the community. The potential impact of Mediterranean diet, hypertension control and decreasing vascular risk factors is discussed.

Incidence of spinal cord injury worldwide: a systematic review.

BACKGROUND: Incidence studies of spinal cord injury (SCI) are important for health-care planning and epidemiological research. This review gives a quantitative update on SCI epidemiology worldwide through a statistical evaluation of incidence rates. METHODS: A systematic review was conducted. For each study, the crude rate ratio was calculated and, when possible, age- and gender-adjusted incidence rate ratios with 95% CI were determined by direct adjustment or using Poisson regression. RESULTS: Thirteen studies were included. Annual crude incidence rates in traumatic SCI varied from 12.1 per million in The Netherlands to 57.8 per million in Portugal. Compared to the Portuguese reference study, incidence rates showed a 3-fold variation, with the highest rates in Canada and Portugal. Most traumatic SCI studies showed a bimodal age distribution. The first peak was found in young adults between 15 and 29 years and a second peak in older adults (mostly > or = 65 years). Motor vehicle accidents and falls were the most prevalent causes of injury accounting for nearly equal percentages. In contrast, another age pattern in non-traumatic SCI reflected steadily increasing incidence with advancing age. CONCLUSIONS: The results show significant variation in SCI incidence with changing epidemiological patterns. A trend towards increased incidence in the elderly was observed, likely due to falls and non-traumatic injury.

Independent validation of the scales for outcomes in Parkinson's disease-autonomic (SCOPA-AUT).

BACKGROUND AND PURPOSE: Autonomic dysfunction is common in Parkinson's disease (PD) and causes a great impact in health-related quality of life (HRQL) and functional status of patients. This study is the first independent validation of the Scales for Outcomes in PD-Autonomic (SCOPA-AUT). METHODS: In an observational, cross-sectional study (ELEP Study), 387 PD patients were assessed using, in addition to the SCOPA-AUT, the Hoehn and Yahr staging, SCOPA-Motor, SCOPA-Cognition, Cumulative Illness Rating Scale-Geriatrics, modified Parkinson Psychosis Rating Scale, Clinical Impression of Severity Index for PD, Hospital Anxiety and Depression Scale, SCOPA-Sleep, SCOPA-Psychosocial, pain and fatigue visual analogue scales, and EQ-5D. SCOPA-AUT acceptability, internal consistency, construct validity, and precision were explored. RESULTS: Data quality was satisfactory (97%). SCOPA-AUT total score did not show floor or ceiling effect, and skewness was 0.40. Cronbach's alpha coefficients ranged from 0.64 (Cardiovascular and Thermorregulatory subscales) to 0.95 (Sexual dysfunction, women). Item homogeneity index was low (0.24) for Gastrointestinal subscale. Factor analysis identified eight factors for men (68% of the variance) and seven factors for women (65% of the variance). SCOPA-AUT correlated at a high level with specific HRQL and functional measures (r(S) = 0.52-0.56). SCOPA-AUT scores were higher for older patients, for more advanced disease, and for patients treated only with levodopa (Kruskal-Wallis test, P < 0.01). Standard error of measurement for SCOPA-AUT subscales was 0.81 (sexual, men) - 2.26 (gastrointestinal). CONCLUSIONS: Despite its heterogeneous content, which determines some weaknesses in the psychometric attributes of its subscales, SCOPA-AUT is an acceptable, consistent, valid and precise scale.

Atributos psicométricos de la Scales for Outcomes in Parkinson’s Disease-Psychosocial (SCOPA-PS): validación en España y revisión / Atributos psicométricos de la scales for outcomes in Parkinson's disease-psychosocial (SCOPA-PS): Validación en España y Revisión

Resumen. Objetivos. Evaluar los atributos psicométricos de la Scales for Outcomes in Parkinson’s Disease-Psychosocial (SCOPA-PS) en España, y compararlos con los estudios previos. Pacientes y métodos. Estudio multicéntrico y transversal sobre 387 pacientes con enfermedad de Parkinson (EP), el 70% en estadios Hoehn y Yahr (HY) 2 o 3, con edad media 65,8 ± 11,1 años y 8,1 ± 6 años de evoluación. Se aplicaron las siguientes medidas: SCOPA-Motor, SCOPA-Cognición, Parkinson’s Psychosis Rating Scale modificada, índice de impresión clínica de gravedad (CISI-PD), Cumulative Illness Rating Scale- Geriatrics, SCOPA-Autonómica, SCOPA-Sueño, escala hospitalaria de ansiedad y depresión (HADS), escalas visuales analógicas de dolor y fatiga, EQ-5D y SCOPA-PS. Se analizaron la aceptabilidad, consistencia interna, dimensionalidad, validez de constructo y precisión de la SCOPA-PS. Resultados. El índice sumarial de la SCOPA-PS no mostró efecto suelo o techo. La consistencia interna fue satisfactoria (alfa = 0,85; correlación ítem-total => 0,39). Se identificaron dos factores (un 53,5% de la varianza). La SCOPA-PS mostró una alta correlación (rS => 0,50) con el índice EQ-5D, HADS, SCOPA-Motor y SCOPAAutonómica, y moderada (rS = 0,35-0,49) con CISI-PD, fatiga y HY. La SCOPA-PS discriminó significativamente entre pacientes agrupados por estadios HY, niveles de gravedad del CISI-PD, grupos de edad y de duración de la EP. El error estándar de la medida resultó 7,24 ± 18,7. En conjunto, estos hallazgos concuerdan con estudios previos. Conclusión. La SCOPAPS es una escala con aceptabilidad satisfactoria, consistente, válida y precisa para evaluar el impacto psicosocial de la EP (AU)

Summary. Aims. To evaluate the psychometric attributes of the Scales for Outcomes in Parkinson’s Disease-Psychosocial (SCOPA-PS) in Spain and to compare them with previous studies. Patients and methods. We performed a multi-centre, crosssectional study of 387 patients with Parkinson’s disease (PD), 70% of whom were in Hoehn and Yahr (HY) stages 2 or 3, with a mean age of 65.8 ± 11.1 years and 8.1 ± 6 years’progression. The following measures were applied: SCOPA-Motor, SCOPACognition, modified Parkinson’s Psychosis Rating Scale, Clinical Impression of Severity Index for Parkinson’s Disease (CISIPD), Cumulative Illness Rating Scale-Geriatrics, SCOPA-Autonomic, SCOPA-Sleep, Hospital Anxiety and Depression Scale (HADS), Fatigue and Pain Visual Analogue Scales, EQ-5D and SCOPA-PS. Acceptability, internal consistence, dimensionality, construct validity and precision of the SCOPA-PS were analysed. Results. The SCOPA-PS summary index displayed no ceiling or floor effect. Internal consistence was satisfactory (alpha = 0.85; item-total correlation => 0.39). Two factors were identified (53.5% of the variance). The SCOPA-PS was highly correlated (rS => 0.5) with the HADS, SCOPA-Motor, SCOPA-Automatic and EQ-5D index, and moderately so (rS = 0.35-0.49) with CISI-PD, fatigue and HY. The SCOPA-PS discriminated significantly between patients grouped according to HY stages, levels of severity of the CISI-PD, age groups and length of time with PD. The standard error of the measure was 7.24 ± 18.7. As a whole, these findings are in agreement with previous studies. Conclusions. The SCOPA-PS is a scale with a satisfactory degree of acceptability, and it is consistent, valid and precise for evaluating the psychosocial impact of PD (AU)

[Psychometric attributes of the Scales for Outcomes in Parkinson's Disease-Psychosocial (SCOPA-PS): validation in Spain and review]. / Atributos psicométricos de la Scales for Outcomes in Parkinson's Disease-Psychosocial (SCOPA-PS): validación en España y revisión.

AIMS: To evaluate the psychometric attributes of the Scales for Outcomes in Parkinson's Disease-Psychosocial (SCOPA-PS) in Spain and to compare them with previous studies. PATIENTS AND METHODS: We performed a multi-centre, cross-sectional study of 387 patients with Parkinson's disease (PD), 70% of whom were in Hoehn and Yahr (HY) stages 2 or 3, with a mean age of 65.8 +/- 11.1 years and 8.1 +/- 6 years' progression. The following measures were applied: SCOPA-Motor, SCOPA-Cognition, modified Parkinson's Psychosis Rating Scale, Clinical Impression of Severity Index for Parkinson's Disease (CISI-PD), Cumulative Illness Rating Scale-Geriatrics, SCOPA-Autonomic, SCOPA-Sleep, Hospital Anxiety and Depression Scale (HADS), Fatigue and Pain Visual Analogue Scales, EQ-5D and SCOPA-PS. Acceptability, internal consistence, dimensionality, construct validity and precision of the SCOPA-PS were analysed. RESULTS: The SCOPA-PS summary index displayed no ceiling or floor effect. Internal consistence was satisfactory (alpha = 0.85; item-total correlation => 0.39). Two factors were identified (53.5% of the variance). The SCOPA-PS was highly correlated (r(S) => 0.5) with the HADS, SCOPA-Motor, SCOPA-Automatic and EQ-5D index, and moderately so (r(S) = 0.35-0.49) with CISI-PD, fatigue and HY. The SCOPA-PS discriminated significantly between patients grouped according to HY stages, levels of severity of the CISI-PD, age groups and length of time with PD. The standard error of the measure was 7.24 +/- 18.7. As a whole, these findings are in agreement with previous studies. CONCLUSIONS: The SCOPA-PS is a scale with a satisfactory degree of acceptability, and it is consistent, valid and precise for evaluating the psychosocial impact of PD.

[Psychometric attributes of Scales for Outcomes in Parkinson's Disease-Cognition (SCOPA-Cog), Castilian language]. / Atributos psicométricos de la Scales for Outcomes in Parkinson's Disease-Cognition (SCOPA-Cog), versión en Castellano.

AIM: To test the psychometric attributes of the Scales for Outcomes in Parkinson's Disease-Cognition (SCOPA-Cog), in Castilian language. PATIENTS AND METHODS: It is a multicenter, cross-sectional study carried out on 387 Parkinson's disease (PD) patients. They were 70% in Hoehn and Yahr stages 2 or 3; their mean age was 65,8 years and they underwent the disease for 8,1 years. Rater-based -SCOPA-Motor, modified Parkinson's Psychosis Rating Scale, Clinical Impression of Severity Index for PD (CISI-PD), Cumulative Illness Rating Scale-Geriatrics- and self-administered -SCOPA-Autonomic, SCOPA-Sleep, SCOPA-Psychosocial, Hospital Anxiety and Depression Scale, EuroQoL- assessments were applied. For SCOPA-Cog, the following psychometric attributes were analysed: acceptability, internal consistency, dimensionality, construct validity, and precision. A cut-off point for dementia and SCOPA-Cog score's predictors were explored. RESULTS: SCOPA-Cog was free from floor and ceiling effect. The internal consistency was satisfactory (alpha = 0,83) and the item-total correlation resulted equal or upper than 0,45. Two factors were identified (52% of variance), one of them formed by 3 out of the 4 memory-related items. The correlation with other measures was weak (rS < 0,35), except for the CISI-PD's item 'cognitive state' (rS = 0,51). SCOPA-Cog scored significantly different for Hoehn and Yahr stages and for patients grouped by age, age at onset of PD, and education. The standard error of measurement was 3,02. A cut-off point 19/20 reached 76% sensitivity and specificity for dementia. Age and age at onset of PD resulted the strongest predictors. CONCLUSION: SCOPA-Cog is a consistent, valid, and precise measure for assessment of the cognitive disorder in PD.

[Preventing dependency in the elderly]. / Prevención de la dependencia en las personas mayores.

INTRODUCTION: Dependency, i.e. the need to depend on another person to perform activities of daily living, is the main concern and cause of suffering and poor quality of life in the elderly. The prevalence of dependency increases with age and is related to the presence of prior disease and fragility. Dependency is associated with increased morbidity, mortality and institutionalization, as well as with greater health and social resource utilization, all of which increases health costs. OBJECTIVE: To create a consensus document on the main health recommendations for the prevention of dependency in the elderly, based on the scientific evidence available to date, with the collaboration of scientific societies and public health administrations (the Spanish Ministry of Health, Autonomous Communities and Cities). METHODS: a) a preliminary consensus document was drafted by an expert group composed of representatives of various scientific societies and health administrations. This document was based on a review of the recommendations and guidelines published by the main organizations involved in health promotion and the prevention of disease, functional deterioration and dependency in the elderly; b) the consensus document was reviewed by the remaining experts assigned by the scientific societies and central and autonomous administrations; c) the final document was approved after a session in which the text was discussed and reviewed by all the experts participating in the working group (including the academic committee); d) the document was presented and discussed in the First National Conference on Prevention and Health Promotion in Clinical Practice in Spain. All participating experts signed a conflicts of interest statement. RESULTS: The document provides recommendations, with their grades of evidence, grouped in the following three categories: a) health promotion and disease prevention, with specific preventive activities for the elderly, including prevention of geriatric syndromes; b) prevention of functional deterioration, with clinical recommendations that can be applied in primary and specialized care; c) prevention of iatrogeny (drug prescription, inappropriate use of diagnostic and therapeutic modalities and healthcare). These recommendations were tailored to the characteristics of the older person (OP), categorized in five groups: healthy OP, OP with chronic disease, fragile or at risk OP, dependent OP, and OP at the end of life. CONCLUSION: These recommendations should be implemented by public health administrations to improve strategies for the prevention of dependency in the elderly in the xxi century.

[Usefulness of the 14-3-3 test for the diagnosis of sCJD evaluated by a Spanish reference center]. / Utilidad del inmunoensayo de la proteína 14-3-3 en el diagnóstico de la enfermedad de Creutzfeldt-Jakob examinada desde un centro de referencia nacional.

INTRODUCTION: The performance of the 14-3-3 protein test has been shown to be adequate for sporadic Creutzfeldt-Jakob disease (sCJD) diagnosis in selected populations, but its routine validity has been questioned. METHODS: One thousand and sixty-eight patients with clinically suspected sCJD were analyzed in a Spanish reference center. In order to explore the influence of the clinical context on the performance of the immunoassay, the patients were classified at sample reception according to the World Health Organization (WHO) diagnostic criteria excluding the 14-3-3 test results. The yield of the immunoassay was evaluated in each subgroup with criteria of probable, possible sCJD or non-sCJD. RESULTS: In the set of patients with suspicion of sCJD the inclusion of the 14-3-3 test produces a significant increase in the diagnosis certainty (positive likelihood ratio: 10.1) compared to the WHO's criteria, excluding the 14-3-3 test. For patients classified at sample reception as probable sCJD (n=166), possible sCJD (n=129) and non-sCJD (n=773), the positive predictive values for the test were 98.4%, 97.5% and 31%, and the negative predictive values were 22.2%, 73.4% and 100%, respectively. CONCLUSIONS: The predictive values of the assay vary according to the previous diagnostic certainty. Therefore, in order to interpret correctly the test, it is necessary to evaluate the degree of initial clinical suspicion of the patient at the moment of the cerebrospinal fluid (CSF) extraction. This study offers up-to-date information, referenced to the Spanish population, and in useful format, and it is intended to serve as a guideline for 14-3-3 test results interpretation to the clinicians in our community.

Prevalence of dementia subtypes in El Prat de Llobregat, Catalonia, Spain: the PRATICON study.

BACKGROUND: Studies on dementia subtypes show a wide variation in the prevalence of Alzheimer's disease (AD) and vascular dementia (VD) worldwide. However, studies reporting on Lewy body dementia (LBD) and frontotemporal dementia (FTD) are sparse. AIMS: To describe the prevalence of dementia and subtypes. METHOD: A 34% sample of 5,150 subjects aged 70 years and over in El Prat de Llobregat (Barcelona) were screened by the Mini-Mental State Examination. When scoring <24, participants were assessed to establish a diagnosis. RESULTS: There were 165 subjects diagnosed with dementia (prevalence of 9.4%). Subtypes of dementia were: AD 69.1%, VD 12.7%, LBD 9.1%, FTD 3% and secondary dementia 1.8%. Prevalences were: AD 6.5%, VD 1.2%, LBD 0.9% and FTD 0.3%. CONCLUSIONS: AD and VD were the most common type of dementia. Prevalence of dementia, AD and FTD were similar to those reported, while prevalence of VD and LBD were lower.

Health-related quality of life in a population-based sample of people with multiple sclerosis in Stockholm County.

The aim of this descriptive, cross-sectional study was to analyse health-related quality of life (HRQoL) in a population-based sample of people with multiple sclerosis (PwMS) in Stockholm County, with respect to disease-related and sociodemographic factors and coping capacity. A further aim was to compare our results on HRQoL--collected by face-to-face interviews at home-visits--with the results from a study with a mail-surveyed sample of PwMS in Stockholm. Home visits were made to 166 PwMS with clinically definite MS. Data were obtained from structured interviews using the Sickness Impact Profile (SIP), EuroQol-5D (EQ-5D) and the Sense of Coherence Scale. HRQoL was widely affected, especially in home management, walking and recreation. Self-rated HRQoL was worse in PwMS, including those with milder disease and shorter disease duration, than in the general population. Factors that were independently associated with large impact on HRQoL were disease severity, work status and coping capacity. The EQ-5D score of PwMS was more favourable when assessed by face-to face interviews at home in our study than in the study using mailed questionnaires. The study gives detailed information on HRQoL and will contribute to the base needed for organizing health care services aimed at improving HRQoL of PwMS in Stockholm.

Classification of surgical procedures for epidemiologic assessment of sporadic Creutzfeldt-Jakob disease transmission by surgery.

BACKGROUND: In this preparatory phase of a case-control study, we propose and evaluate a new tool for classifying surgical procedures (SPs) in categories useful for epidemiologic research on surgical transmission of sporadic Creutzfeldt-Jakob disease (sCJD). METHODS: All SPs reported to the Swedish National Hospital Discharge Registry in the period 1974-2002, and undergone by 212 Swedish patients with registered diagnosis of CJD at death, hospital discharge or notification, in the period 1987-2002, 1060 age-, sex- and residence-matched controls and 1340 randomly chosen population controls, were reclassified into one of six categories of hypothetical transmission risk level. For that purpose the following two attributes were used: non-disposable instruments involved; and highest assigned ad-hoc risk level for four tissues or anatomical structures contacting such instruments. RESULTS: A total of 1170 different SP codes were reclassified as follows: 3.1% in the high-risk, 59.1% in the lower-risk, 24.4% in the lowest-risk, and 2.1% in the no-risk groups, with 11.3% procedures negatively defined by rubric as "other than..." being assigned to two spurious diluted-high and diluted-lower risk categories. The high-risk group mainly comprised neurosurgical (53%) and ophthalmic (39%) procedures. Sensitivity of neurosurgery and of ophthalmic surgery excluding neurosurgery, for the high- and diluted-high risk vs. other categories was 46% and 84%, while specificity was 98% and 95%, respectively. Sensitivity analysis based on these indices revealed that non-significant odds ratio effects of 1.4 and 1.3 for neurosurgery and ophthalmic surgery corresponded to statistically significant values of 5.1 after reclassification. CONCLUSIONS: This classification might contribute to quantify effects masked by use of body-system SP-categories in case-control studies on sCJD transmission by surgery.

Determinants of diagnostic investigation sensitivities across the clinical spectrum of sporadic Creutzfeldt-Jakob disease.

To validate the provisional findings of a number of smaller studies and explore additional determinants of characteristic diagnostic investigation results across the entire clinical spectrum of sporadic Creutzfeldt-Jakob disease (CJD), an international collaborative study was undertaken comprising 2451 pathologically confirmed (definite) patients. We assessed the influence of age at disease onset, illness duration, prion protein gene (PRNP) codon 129 polymorphism (either methionine or valine) and molecular sub-type on the diagnostic sensitivity of EEG, cerebral MRI and the CSF 14-3-3 immunoassay. For EEG and CSF 14-3-3 protein detection, we also assessed the influence of the time point in a patient's illness at which the investigation was performed on the likelihood of a typical or positive result. Analysis included a large subset of patients (n = 743) in whom molecular sub-typing had been performed using a combination of the PRNP codon 129 polymorphism and the form of protease resistant prion protein [type 1 or 2 according to Parchi et al. (Parchi P, Giese A, Capellari S, Brown P, Schulz-Schaeffer W, Windl O, Zerr I, Budka H, Kopp N, Piccardo P, Poser S, Rojiani A, Streichemberger N, Julien J, Vital C, Ghetti B, Gambetti P, Kretzschmar H. Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 1999; 46: 224-233.)] present in the brain. Findings for the whole group paralleled the subset with molecular sub-typing data available, showing that age at disease onset and disease duration were independent determinants of typical changes on EEG, while illness duration significantly influenced positive CSF 14-3-3 protein detection; changes on brain MRI were not influenced by either of these clinical parameters, but overall, imaging data were less complete and consequently conclusions are more tentative. In addition to age at disease onset and illness duration, molecular sub-type was re-affirmed as an important independent determinant of investigation results. In multivariate analyses that included molecular sub-type, time point of the investigation during a patient's illness was found not to influence the occurrence of a typical or positive EEG or CSF 14-3-3 protein result. A typical EEG was most often seen in MM1 patients and was significantly less likely in the MV1, MV2 and VV2 sub-types, whereas VV2 patients had an increased likelihood of a typical brain MRI. Overall, the CSF 14-3-3 immunoassay was the most frequently positive investigation (88.1%) but performed significantly less well in the very uncommon MV2 and MM2 sub-types. Our findings confirm a number of determinants of principal investigation results in sporadic CJD and underscore the importance of recognizing these pre-test limitations before accepting the diagnosis excluded or confirmed. Combinations of investigations offer the best chance of detection, especially for the less common molecular sub-types such as MV2 and MM2.