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1.
Artigo em Russo | MEDLINE | ID: mdl-33081454

RESUMO

OBJECTIVE: To evaluate the frequency of C9orf72-associated frontotemporal dementia (FTD) in the Russian population and to study clinical features of GGGGCC-repeat expansion carriers. MATERIAL AND METHODS: Twenty-eight patients with FTD are included in the study: 15 with a behavioral variant of FTD (bvFTD) and 13 with a agrammatic/non-fluent variant of primary progressive aphasia (avPPA). The mean age was 62 years (34-80), the mean disease duration was 4 years (1-10). The positive family history was noted in 46% of cases. DNA diagnosis was performed using repeat-primed polymerase chain reaction. RESULTS: The frequency of the C9orf72 repeat expansion in patients with FTD was 14%, in patients with bvFTD 20%, in patients with avPPA 8%. The mean age of disease onset in the expansion carriers was 63 (55-75) years. The frequency of the C9orf72 repeats expansion in familial FTD cases was 31%, in sporadic cases 7%. bvFTD with parkinsonian syndrome was noted in two out of four cases, bvFTD with amyotrophic lateral sclerosis (ALS) was shown in one case, avPPA with ALS was shown in one case. One female patient with bvFTD with parkinsonian syndrome presented with cognitive fluctuations that required a differential diagnosis with Lewy body disease. CONCLUSION: This is the first study of the genetic structure of FTD in the Russian population. The prevalence and clinical characteristics of C9orf72-associated FTD were defined, in particular, the spectrum of motor symptoms was shown along with behavioral and aphasic disturbances. DNA diagnosis plays an important role in confirming the diagnosis and selection of patients for potential disease-modifying treatment.


Assuntos
Esclerose Amiotrófica Lateral , Demência Frontotemporal , Adulto , Idoso , Idoso de 80 Anos ou mais , Esclerose Amiotrófica Lateral/diagnóstico , Esclerose Amiotrófica Lateral/epidemiologia , Esclerose Amiotrófica Lateral/genética , Proteína C9orf72/genética , Feminino , Demência Frontotemporal/diagnóstico , Demência Frontotemporal/epidemiologia , Demência Frontotemporal/genética , Humanos , Pessoa de Meia-Idade , Proteínas/genética , Federação Russa/epidemiologia
2.
PLoS One ; 15(8): e0237175, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32790757

RESUMO

Patients' vitamin intake is often not documented and is therefore not considered sufficiently in studies of prescribed medication in patients with amyotrophic lateral sclerosis (ALS). We aimed to determine the prevalence of vitamin use by participants in ALS clinical trials. Data about demographics, disease severity (ALS Functional Rating Scale) and concomitant medication were obtained from the Pooled Resource Open-Access ALS Clinical Trials Database, which contains records from more than 6000 ALS patients who participated in 23 phase II/III clinical trials. Information about vitamin intake for all study subjects was coded into major categories. Clinical data of vitamin users and nonusers were compared, and regression analysis was used to explore the associations among clinical parameters, vitamin use and two measures of disease progression. From the 40.996 available medication records from 6274 subjects, 7338 (17.9%) concerned vitamins. One or more vitamins were used by 3331 subjects (53.1%). Most common was vitamin E, vitamin C and multivitamins. Patients who did and did not take vitamins did not differ in terms of disease progression and ALS Functional Rating Scale score. Patients who took vitamins were younger, were more often female, had a shorter time between onset and diagnosis, had shorter disease duration and more frequently had limb-onset types. Disease progression rate and disease aggressiveness were not associated with vitamin use. Despite unclear evidence, the use of vitamins in ALS is common. However, rapid progression was not observed to be associated with vitamin use.


Assuntos
Esclerose Amiotrófica Lateral/tratamento farmacológico , Uso de Medicamentos/estatística & dados numéricos , Vitaminas/administração & dosagem , Adulto , Idoso , Esclerose Amiotrófica Lateral/epidemiologia , Ensaios Clínicos Fase II como Assunto , Ensaios Clínicos Fase III como Assunto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Vitaminas/uso terapêutico
6.
Adv Exp Med Biol ; 1195: 179-187, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32468476

RESUMO

Amyotrophic lateral sclerosis (ALS) is a rare, neurodegenerative disease that affects the human motor system. ALS is a highly heterogeneous disease, depending on several causative factors. The heterogeneity of the disease is also reflected in the variation of the symptoms in ALS patients. The worldwide annual incidence of ALS is about 2.08 per 100,000 with uniform rates in Caucasian populations and lower rates in African, Asian, and Hispanic populations, while the number of individuals with ALS is expected to grow significantly between 2015 and 2040 with an estimated increase of 69% (Chio et al. 2013a; Arthur et al. 2016).


Assuntos
Esclerose Amiotrófica Lateral/diagnóstico , Esclerose Amiotrófica Lateral/metabolismo , Biomarcadores/análise , Esclerose Amiotrófica Lateral/epidemiologia , Biomarcadores/metabolismo , Grupos de Populações Continentais/estatística & dados numéricos , Humanos , Incidência
8.
Artigo em Inglês | MEDLINE | ID: mdl-32331298

RESUMO

Objectives: Amyotrophic lateral sclerosis (ALS) is a progressive and fatal neurodegenerative disease with still unknown etiology. We aimed at investigating the association between environmental and occupational factors with ALS risk. Methods: We performed a population-based case-control study in four Italian provinces (Catania, Modena, Novara, and Reggio Emilia) by administration of tailored questionnaires to ALS cases (n = 95) and randomly selected population referents (n = 135). We estimated ALS risk by calculating the odds ratio (OR) with its 95% confidence interval (CI) using an unconditional logistic regression model. Results: We found a positive association with disease risk for history of occupation in the agricultural sector (OR = 2.09, 95% CI 0.79-7.54), especially for longer than 10 years (OR = 2.72, 95% 1.02-7.20). Overall occupational exposure to solvents also suggested a positive association, especially for thinners (OR = 2.27, 95% CI 1.14-4.54) and paint removers (OR = 2.01, 95% CI 0.90-4.48). Both occupational and environmental exposure to electromagnetic fields show a slightly increased risk with OR = 1.69 (95% CI 0.70-4.09) and 2.41 (95% CI 1.13-5.12), respectively. Occupational but not environmental exposure to pesticides (OR = 1.22, 95% CI 0.63-2.37), particularly fungicides, and exposure to metals (OR = 4.20, 95% CI 1.88-9.38), particularly lead, mercury, and selenium, showed an imprecise but positive association. Finally, there was an indication of increased risk for living in proximity to water bodies. Conclusions: Despite the caution that needs to be used due to some study limitations, such as the low number of exposed subjects and the possibility of recall bias, these results suggest the potential role of some environmental and occupational factors in ALS etiology.


Assuntos
Esclerose Amiotrófica Lateral , Doenças Neurodegenerativas , Exposição Ocupacional , Idoso , Esclerose Amiotrófica Lateral/epidemiologia , Estudos de Casos e Controles , Feminino , Humanos , Itália , Masculino , Pessoa de Meia-Idade , Fatores de Risco
9.
Environ Res ; 186: 109530, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32335431

RESUMO

BACKGROUND: Epidemiological studies highlighted the possibility that exposure to cyanotoxins leads to the development of the neurodegenerative disease amyotrophic lateral sclerosis (ALS). METHODS: We devised a population-based case-control study in two Italian populations. We used residential proximity of the residence to water bodies as a measure of possible exposure to cyanotoxins. RESULTS: Based on 703 newly-diagnosed ALS cases and 2737 controls, we calculated an ALS odds ratio (OR) of 1.41 (95% CI: 0.72-2.74) for current residence in the vicinity of water bodies, and a slightly lower estimate for historical residence (OR: 1.31; 95% CI: 0.57-2.99). Subjects <65 years and people living in the Northern Italy province of Modena had higher ORs, especially when historical residence was considered. CONCLUSIONS: Overall, despite some risk of bias due to exposure misclassification and unmeasured confounding, our results appear to support the hypothesis that cyanotoxin exposure may increase ALS risk.


Assuntos
Esclerose Amiotrófica Lateral , Cianobactérias , Doenças Neurodegenerativas , Esclerose Amiotrófica Lateral/induzido quimicamente , Esclerose Amiotrófica Lateral/epidemiologia , Estudos de Casos e Controles , Humanos , Itália/epidemiologia , Fatores de Risco
10.
Neurology ; 94(14): e1452-e1459, 2020 04 07.
Artigo em Inglês | MEDLINE | ID: mdl-32188763

RESUMO

OBJECTIVE: An estimated 1.4 million young caregivers (<19 years of age) in the United States provide care to ill family members yet remain hidden from state and national caregiving programs and services, including amyotrophic lateral sclerosis (ALS) caregiving services. Given the intensive care needs and acuity of ALS, appreciation of the young caregiver experience within the family context may have a significant impact on patient and family quality of life. This article seeks to identify family and youth caregiver characteristics and perceptions of care through interviews with 38 youth caregivers and their families with ALS. METHODS: Online adult surveys and follow-up youth interviews were conducted with families with ALS across the United States in this cross-sectional study. Participants were accessed through chapters of the ALS Association. Both thematic content analysis and descriptive statistics were used. RESULTS: Youth caregivers (n = 38) ranged in age from 8 to 18 years and spent an average of 5 h/d providing care for an average of 12 tasks. Persons with ALS relied on youth primarily due to cost and identified complex feelings about relying on youth caregivers, including feeling like a failure, guilty, but proud. CONCLUSION: Youth are intricately involved in all areas of caregiving in ALS. They are isolated and have little training or guidance in care, yet they are able to identify ways to manage their care burden. Results provide clear implications for health care professionals in designing best care and support practices for persons with ALS and their young caregivers.


Assuntos
Esclerose Amiotrófica Lateral/epidemiologia , Cuidadores/estatística & dados numéricos , Adaptação Psicológica , Adolescente , Adulto , Cuidadores/economia , Criança , Custos e Análise de Custo , Estudos Transversais , Família , Feminino , Culpa , Humanos , Masculino , Pessoa de Meia-Idade , Qualidade de Vida , Fatores Socioeconômicos , Inquéritos e Questionários , Estados Unidos/epidemiologia
11.
Eur J Epidemiol ; 35(3): 241-249, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32193761

RESUMO

Studies of occupational solvent exposures and amyotrophic lateral sclerosis (ALS) have been conflicting. We conducted a population-based case-control study of mixed occupational solvent exposures and ALS. Using the Danish National Patient Registry, we identified ALS cases in Denmark from 1982 to 2013, and matched them to 100 controls based on sex and birth year. We estimated cumulative exposures to solvents (benzene, methylene chloride, toluene, trichloroethylene, perchloroethylene, and 1,1,1-trichloroethane) via job exposure matrices and applied them to occupational history from the Danish Pension Fund. Sex-stratified conditional logistic regression analyses revealed higher adjusted odds of ALS for men with exposure to benzene (aOR = 1.20; 95% CI 1.02, 1.41) and methylene chloride (aOR = 1.23; 95% CI 1.07, 1.42). We used weighted quantile sum regression to explore combined solvent exposures and risk of ALS in exposed subjects and found increased odds of 26 to 28% in all exposure lag periods for every one-unit increase in the mixture index in men. Weights of methylene chloride predominated the mixture index in all lag periods. Our study suggests an increased risk of ALS in men exposed to multiple solvents, with the greatest influence being from methylene chloride. These findings highlight the need to utilize mixtures analysis when considering co-occurring exposures.


Assuntos
Esclerose Amiotrófica Lateral/induzido quimicamente , Doenças Profissionais/induzido quimicamente , Exposição Ocupacional/efeitos adversos , Solventes/efeitos adversos , Adulto , Idoso , Esclerose Amiotrófica Lateral/epidemiologia , Estudos de Casos e Controles , Dinamarca/epidemiologia , Feminino , Humanos , Masculino , Cloreto de Metileno/efeitos adversos , Pessoa de Meia-Idade , Doenças Profissionais/epidemiologia , Vigilância da População , Fatores de Risco
12.
Muscle Nerve ; 62(2): 187-191, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32134532

RESUMO

Genetic, epigenetic, and environmental factors are relevant in the causation of amyotrophic lateral sclerosis (ALS) in a multistep cascade. We suggest that exposure to environmental pollutants in early life is one such factor. ALS was first described in the 19th century in the context of the Industrial Revolution that began more than 50 years earlier. The rising incidence of ALS thereafter correlates with increasing longevity, but this is an incomplete association. We suggest that increasing exposure to environmental pollutants due to industrial activity, acting over a lifetime, is also important. The combination of genetic mutations and pollutant exposure, with increased life expectancy, may account for the apparent variations in incidence of the disease in different countries and continents and even regionally within a given country. This hypothesis is testable by focused epidemiological studies, evaluating early and lifelong industrial pollutant exposure of differing types, within the Bradford Hill framework.


Assuntos
Esclerose Amiotrófica Lateral/epidemiologia , Exposição Ambiental/estatística & dados numéricos , Poluentes Ambientais , Interação Gene-Ambiente , Desenvolvimento Industrial/estatística & dados numéricos , Expectativa de Vida , Esclerose Amiotrófica Lateral/genética , Esclerose Amiotrófica Lateral/história , Proteína C9orf72/genética , Causalidade , Proteínas de Ligação a DNA/genética , Exposição Ambiental/história , História do Século XIX , História do Século XX , História do Século XXI , Humanos , Incidência , Desenvolvimento Industrial/história , Mutação , Superóxido Dismutase-1/genética
13.
J Neurol Neurosurg Psychiatry ; 91(5): 520-525, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-32139654

RESUMO

OBJECTIVE: Amyotrophic lateral sclerosis (ALS) is a severe neurodegenerative disease and information on disease burden of ALS in mainland China was limited. We aimed to estimate the prevalence and incidence of ALS in China. METHODS: We used 2012-2016 data from China's Urban Employee Basic Medical Insurance and Urban Residence Basic Medical Insurance, covering approximately 0.43 billion individuals. ALS cases were identified by the primary diagnosis (International Classification of Diseases code or text of diagnosis) in the insurance database. RESULTS: The crude prevalence and incidence in 2016 were 2.91 per 100 000 person-years (95% CI 2.31 to 3.58) and 1.65 (95% CI 1.33 to 2.01), respectively. The standardised prevalence and incidence based on 2010 Chinese census data were 2.97 (95% CI 2.91 to 3.03) and 1.62 (95% CI 1.58 to 1.67), respectively. The annual prevalence between 2013 and 2016 remained relatively constant, ranging from 2.91 (95% CI 2.31 to 3.58) in 2016 to 3.29 (95% CI 2.51 to 4.17) in 2014 (linear regression: ß=-0.129, p=0.104). Both rates peaked in the group aged 75-79 years. CONCLUSIONS: The prevalence and incidence of ALS in mainland China were lower than those in developed countries, and maintained a relatively stable trend. The age at onset and age at diagnosis for ALS patients were younger than those in developed countries. Further research is expected to clarify the potential pathophysiological mechanism of ALS.


Assuntos
Esclerose Amiotrófica Lateral/epidemiologia , População Urbana/estatística & dados numéricos , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , China/epidemiologia , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Prevalência , Fatores Sexuais , Adulto Jovem
14.
Rev Neurol (Paris) ; 176(5): 301-315, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32147204

RESUMO

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease with no known cure. Approximately 90% of ALS cases are sporadic, suggesting there are multiple contributing factors that influence the disease risk, onset, and progression. Diet and sex are two factors that have been reported to alter ALS risk, onset and progression in humans and in animal models, providing potential modifiers of disease. Several epidemiological studies have identified diets that positively affect ALS patients, including various high-calorie fat or sugar-based diets, while animal models have been developed to test how these diets are working on a molecular level. These diets may offset the metabolic alterations that occur in ALS, such as hypermetabolism, lowered body mass index(BMI), and hyperlipidemia. Sex-dependent differences have also come forth from large-scale epidemiological studies as well as mouse-model studies. In addition, sex hormones have been shown to affect disease risk or progression. Herein, studies on the effects of diet and sex on ALS risk, onset, and progression will be reviewed. Understanding these diet- and sex-dependent outcomes may lead to optimized patient-specific therapies for ALS.


Assuntos
Esclerose Amiotrófica Lateral/epidemiologia , Esclerose Amiotrófica Lateral/etiologia , Dieta , Caracteres Sexuais , Esclerose Amiotrófica Lateral/patologia , Animais , Índice de Massa Corporal , Modelos Animais de Doenças , Progressão da Doença , Feminino , Hormônios Esteroides Gonadais/sangue , Hormônios Esteroides Gonadais/fisiologia , Humanos , Masculino , Camundongos
15.
Neurology ; 94(15): e1657-e1663, 2020 04 14.
Artigo em Inglês | MEDLINE | ID: mdl-32071166

RESUMO

OBJECTIVE: To establish whether amyotrophic lateral sclerosis (ALS) is a multistep process in South Korean and Japanese populations when compared to Australian cohorts. METHODS: We generated incident data by age and sex for Japanese (collected between April 2009 and March 2010) and South Korean patients with ALS (collected between January 2011 and December 2015). Mortality rates were provided for Australian patients with ALS (collected between 2007 and 2016). We regressed the log of age-specific incidence against the log of age with least squares regression for each ALS population. RESULTS: We identified 11,834 cases of ALS from the 3 populations, including 6,524 Australian, 2,264 Japanese, and 3,049 South Korean ALS cases. We established a linear relation between the log incidence and log age in the 3 populations: Australia r 2 = 0.99, Japan r 2 = 0.99, South Korea r 2 = 0.99. The estimate slopes were similar across the 3 populations, being 5.4 (95% confidence interval [CI], 4.8-5.5) in Japanese, 5.4 (95% CI, 5.2-5.7) in Australian, and 4.4 (95% CI, 4.2-4.8) in South Korean patients. CONCLUSIONS: The linear relationship between log age and log incidence is consistent with a multistage model of disease, with slope estimated suggesting that 6 steps were required in Japanese and Australian patients with ALS while 5 steps were needed in South Korean patients. Identification of these steps could identify novel therapeutic strategies.


Assuntos
Esclerose Amiotrófica Lateral/epidemiologia , Esclerose Amiotrófica Lateral/terapia , Progressão da Doença , Adulto , Idoso , Austrália , Grupos de Populações Continentais , Feminino , Humanos , Incidência , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , República da Coreia/epidemiologia
16.
Artigo em Inglês | MEDLINE | ID: mdl-32019087

RESUMO

Background: Amyotrophic lateral sclerosis (ALS) is a progressive, fatal neurodegenerative disease of the motor neurons. The etiology of ALS remains largely unknown, particularly with reference to the potential environmental determinants. Methods: We performed a population-based case-control study in four provinces from both Northern and Southern Italy in order to assess non-genetic ALS risk factors by collecting through tailored questionnaires information about clinical and lifestyle factors. We estimated ALS risk by calculating odds ratio (OR) with its 95% confidence interval (CI) using unconditional logistic regression models adjusted for sex, age and educational attainment. Results: We recruited 230 participants (95 cases and 135 controls). We found a possible positive association of ALS risk with trauma, particularly head trauma (OR = 2.61, 95% CI 1.19-5.72), electric shock (OR = 2.09, 95% CI 0.62-7.06), and some sports, although at a competitive level only. In addition, our results suggest an increased risk for subjects reporting use of private wells for drinking water (OR = 1.38, 95% CI 0.73-2.27) and for use of herbicides during gardening (OR = 1.95, 95% CI 0.88-2.27). Conversely, there was a suggestion of an inverse association with overall fish consumption (OR = 0.27, 95% CI 0.12-0.60), but with no dose-response relation. Consumption of some dietary supplements, namely those containing amino acids and, in the Southern Italy population, vitamins and minerals such as selenium, seemed associated with a statistically imprecise increased risk. Conclusions: Our results suggest a potential etiologic role a number of clinical and lifestyle factors with ALS risk. However, caution is needed due to some study limitations. These include the small sample size and the low number of exposed subjects, which affect statistical precision of risk estimates, the potential for exposure misclassification, and the uncertainties about mechanisms underpinning the possible association between these factors and disease risk.


Assuntos
Esclerose Amiotrófica Lateral/etiologia , Estilo de Vida , Fatores de Risco , Idoso , Esclerose Amiotrófica Lateral/epidemiologia , Estudos de Casos e Controles , Feminino , Humanos , Itália/epidemiologia , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Razão de Chances , Saúde da População , Projetos de Pesquisa , Selênio
17.
Neurology ; 94(8): e802-e810, 2020 02 25.
Artigo em Inglês | MEDLINE | ID: mdl-31907290

RESUMO

OBJECTIVE: To assess the determinants of amyotrophic lateral sclerosis (ALS) phenotypes in a population-based cohort. METHODS: The study population included 2,839 patients with ALS diagnosed in Piemonte, Italy (1995-2015). Patients were classified according to motor (classic, bulbar, flail arm, flail leg, predominantly upper motor neuron [PUMN], respiratory) and cognitive phenotypes (normal, ALS with cognitive impairment [ALSci], ALS with behavioral impairment [ALSbi], ALSci and ALSbi combined [ALScbi], ALS-frontotemporal dementia [FTD]). Binary logistic regression analysis was adjusted for sex, age, and genetics. RESULTS: Bulbar phenotype correlated with older age (p < 0.0001), women were more affected than men at increasing age (p < 0.0001), classic with younger age (p = 0.029), men were more affected than women at increasing age (p < 0.0001), PUMN with younger age (p < 0.0001), flail arm with male sex (p < 0.0001) and younger age (p = 0.04), flail leg with male sex with increasing age (p = 0.008), and respiratory with male sex (p < 0.0001). C9orf72 expansions correlated with bulbar phenotype (p < 0.0001), and were less frequent in PUMN (p = 0.041); SOD1 mutations correlated with flail leg phenotype (p < 0.0001), and were less frequent in bulbar (p < 0.0001). ALS-FTD correlated with C9orf72 (p < 0.0001) and bulbar phenotype (p = 0.008), ALScbi with PUMN (p = 0.014), and ALSci with older age (p = 0.008). CONCLUSIONS: Our data suggest that the spatial-temporal combination of motor and cognitive events leading to the onset and progression of ALS is characterized by a differential susceptibility to the pathologic process of motor and prefrontal cortices and lower motor neurons, and is influenced by age, sex, and gene variants. The identification of those factors that regulate ALS phenotype will allow us to reclassify patients into pathologically homogenous subgroups, responsive to targeted personalized therapies.


Assuntos
Esclerose Amiotrófica Lateral/classificação , Esclerose Amiotrófica Lateral/epidemiologia , Proteína C9orf72/genética , Disfunção Cognitiva/epidemiologia , Demência Frontotemporal/epidemiologia , Transtornos Motores/epidemiologia , Superóxido Dismutase-1/genética , Fatores Etários , Idoso , Esclerose Amiotrófica Lateral/genética , Disfunção Cognitiva/genética , Comorbidade , Feminino , Demência Frontotemporal/classificação , Demência Frontotemporal/genética , Humanos , Itália/epidemiologia , Masculino , Pessoa de Meia-Idade , Transtornos Motores/classificação , Transtornos Motores/genética , Mutação , Fenótipo , Fatores Sexuais
18.
Brain Nerve ; 71(11): 1129-1137, 2019 Nov.
Artigo em Japonês | MEDLINE | ID: mdl-31722299

RESUMO

This brief review of recent epidemiologic literature and risk factors of sporadic ALS found that the incidence and prevalence of the condition is higher among Caucasians and lower in East Asians, with the Japanese in the middle. The review also found that worldwide, the prevalence increases with age and the condition is 1.3 to 1.6 times higher in males than in females. The number of patients with ALS was calculated based on official diagnostic certificates from fiscal 1997 to 2015 obtained from a registry managed by Japan's Ministry of Health, Labor and Welfare. The data matched with those reported in the guidelines of the Japanese Society of Neurology (2013), with an incidence of 1.1-2.5, and prevalence 7.0-8.5/100,000 people. Smoking has been considered an established risk factor for sporadic ALS. The following possible risk factors have been speculated but are to be confirmed: excessive physical activity, head trauma, farming, environmental pollutants, pesticides, exposure to certain metals/heavy metals, electromagnetic fields, alcohol, low BMI, and low-density lipoprotein. Bandres-Ciga et al. applied linkage disequilibrium score regression and Mendelian randomization to a large data set and concluded that elevated low-density lipoprotein cholesterol is a causal risk factor for ALS.


Assuntos
Esclerose Amiotrófica Lateral/epidemiologia , Humanos , Incidência , Japão , Prevalência , Fatores de Risco
19.
Brain Nerve ; 71(11): 1236-1244, 2019 Nov.
Artigo em Japonês | MEDLINE | ID: mdl-31722309

RESUMO

Recent papers of amyotrophic lateral sclerosis/parkinsonism-dementia complex in the Kii peninsula, Japan (Kii ALS/PDC), published since 2015, were reviewed The studies included transition element of scalp hair analysis, dopaminergic PET study, review of life style changes in the high incident area, neurotoxic BMAA analysis, a clinical report of a migration case, comprehensive neuropathological study, cerebellar tau pathology, nitrative stress in the central nervous system study, optinurin pathology in the spinal cord, and tau PET study. Tau PET was advocated to be a new useful tool for diagnosis, even in the early stage of ALS/PDC with tauopathy. The etiology of Kii ALS/PDC remainds unknown. There are patients and healthy residents within the same environment in the high incidence foci, therefore it is difficult to explain this result by exposure to environmental factors alone. From the genetic viewpoint, rare-disease and rare-variant model may be applied to Kii ALS/PDC. Because there was an immigrant who was diagnosed neuropathologically, and a drastic decrease of the prevalence in the past several decades in the high incident area, it is feasible that Kii ALS/PDC is a multifactorial disease caused by both risk genes and environmental factors. Identifying risk genes and environmental factors for Kii ALS/PDC may contribute to the prevention of neurodegenerative diseases.


Assuntos
Esclerose Amiotrófica Lateral/epidemiologia , Demência/epidemiologia , Transtornos Parkinsonianos/epidemiologia , Humanos , Japão/epidemiologia
20.
Environ Res ; 179(Pt A): 108742, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31629180

RESUMO

Some studies have reported an association between overexposure to selenium and risk of amyotrophic lateral sclerosis (ALS), a rare degenerative disease of motor neurons. From 1986 through 2015, we followed a cohort in Northern Italy that had been inadvertently consuming tap water with unusually high concentrations of inorganic hexavalent selenium from 1974 to 1985. We had previously documented an excess incidence of ALS in this cohort during 1986-1994. Here, we report extended follow-up of the cohort for an additional 21 years, encompassing 50,100 person-years of the exposed cohort and 2,233,963 person-years of the unexposed municipal cohort. We identified 7 and 112 incident ALS cases in the exposed and unexposed cohorts, respectively, yielding crude incidence rates of 14 and 5 cases per 100,000 person-years. A Poisson regression analysis, adjusting for age, sex and calendar year, produced an overall incidence rate ratio (IRR) for ALS of 2.8 (95% confidence interval (CI) 1.3, 6), with a substantially stronger IRR in 1986-1994 (8.2, 95% CI 2.7, 24.7) than in 1995-2015 (1.5, 95% CI 0.5, 4.7), and among women (5.1, 95% CI 1.8, 14.3) than men (1.7, 95% CI 0.5, 5.4). Overall, these results indicate an association between high exposure to inorganic selenium, a recognized neurotoxicant, and ALS incidence, with declining rates after cessation of exposure and stronger effects among women.


Assuntos
Esclerose Amiotrófica Lateral/epidemiologia , Exposição Dietética/estatística & dados numéricos , Água Potável/química , Selênio/análise , Poluentes Químicos da Água/análise , Feminino , Seguimentos , Humanos , Incidência , Itália/epidemiologia , Masculino
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