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      Epidemiology of amyotrophic lateral sclerosis: an update of recent literature

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          Purpose of review

          The cause of amyotrophic lateral sclerosis (ALS) remains unknown for most of the patients with the disease. Epidemiologic studies can help describe disease burden and examine its potential risk factors, providing thereby evidence base for future mechanistic studies. With this review, we aimed to provide a summary of epidemiologic studies published during the past 18 months, which studied the incidence and risk factors for ALS.

          Recent findings

          An increasing incidence and prevalence of ALS continue to be reported from different parts of the world. Several previously studied risk factors are confirmed as causally related to ALS by Mendelian randomization analysis. The previously known prognostic indicators for ALS appear to be the same across populations.

          Summary

          Provided with the increasing number of patients diagnosed with ALS and the improved societal awareness of the disease, more resources should be allocated to the research and care of ALS. Population-based studies, especially population-based disease registers, should be the priorities in ALS research, and more data from outside Europe are needed in gaining a better global perspective of the disease.

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          Most cited references43

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          Causal effects of blood lipids on amyotrophic lateral sclerosis: a Mendelian randomization study

          Abstract Amyotrophic lateral sclerosis (ALS) is a late-onset fatal neurodegenerative disorder that is predicted to increase across the globe by ~70% in the following decades. Understanding the disease causal mechanism underlying ALS and identifying modifiable risks factors for ALS hold the key for the development of effective preventative and treatment strategies. Here, we investigate the causal effects of four blood lipid traits that include high-density lipoprotein, low-density lipoprotein (LDL), total cholesterol and triglycerides on the risk of ALS. By leveraging instrument variables from multiple large-scale genome-wide association studies in both European and East Asian populations, we carry out one of the largest and most comprehensive Mendelian randomization analyses performed to date on the causal relationship between lipids and ALS. Among the four lipids, we found that only LDL is causally associated with ALS and that higher LDL level increases the risk of ALS in both the European and East Asian populations. Specifically, the odds ratio of ALS per 1 standard deviation (i.e. 39.0 mg/dL) increase of LDL is estimated to be 1.14 [95% confidence interval (CI), 1.05–1.24; P = 1.38E-3] in the European population and 1.06 (95% CI, 1.00–1.12; P = 0.044) in the East Asian population. The identified causal relationship between LDL and ALS is robust with respect to the choice of statistical methods and is validated through extensive sensitivity analyses that guard against various model assumption violations. Our study provides important evidence supporting the causal role of higher LDL on increasing the risk of ALS, paving ways for the development of preventative strategies for reducing the disease burden of ALS across multiple nations.
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            Molecular diagnostics of neurodegenerative disorders

            Molecular diagnostics provide a powerful method to detect and diagnose various neurological diseases such as Alzheimer's and Parkinson's disease. The confirmation of such diagnosis allows early detection and subsequent medical counseling that help specific patients to undergo clinically important drug trials. This provides a medical pathway to have better insight of neurogenesis and eventual cure of the neurodegenerative diseases. In this short review, we present recent advances in molecular diagnostics especially biomarkers and imaging spectroscopy for neurological diseases. We describe advances made in Alzheimer's disease (AD), Parkinson's disease (PD), Amyotrophic lateral sclerosis (ALS) and Huntington's disease (HD), and finally present a perspective on the future directions to provide a framework for further developments and refinements of molecular diagnostics to combat neurodegenerative disorders.
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              The changing picture of amyotrophic lateral sclerosis: lessons from European registers

              Prospective population based-registers of amyotrophic lateral sclerosis (ALS) have operated in Europe for over two decades, and have provided important insights into our understanding of ALS. Here, we review the benefits that population registers have brought to the understanding of the incidence, prevalence, phenotype and genetics of ALS and outline the core operating principles that underlie these registers and facilitate international collaboration. Going forward, we offer lessons learned from our collective experience of operating population-based ALS registers in Europe for over two decades, focusing on register design, maintenance, identification and management of bias and the value of cross-national harmonisation and integration.
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                Author and article information

                Journal
                Curr Opin Neurol
                Curr. Opin. Neurol
                CONEU
                Current Opinion in Neurology
                Lippincott Williams & Wilkins
                1350-7540
                1473-6551
                October 2019
                12 August 2019
                : 32
                : 5
                : 771-776
                Affiliations
                Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, Stockholm, Sweden
                Author notes
                Correspondence to Fang Fang, Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, Box 281, 171 77 Stockholm, Sweden. Tel: +46 8 52486131; e-mail: fang.fang@ 123456ki.se
                Article
                WCO320503 00018
                10.1097/WCO.0000000000000730
                6735526
                31361627
                adf20ae9-30df-48ad-84d5-d749563c966a
                Copyright © 2019 The Author(s). Published by Wolters Kluwer Health, Inc.

                This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0

                History
                Categories
                MOTOR NEURON DISEASE: Edited by Albert C. Ludolph
                Custom metadata
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                amyotrophic lateral sclerosis,epidemiology,global perspective,increasing incidence

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