Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues
Amyotrophic lateral sclerosis (ALS) is a relatively rare disease with a reported population incidence of between 1.5 and 2.5 per 100 000 per year. Over the past 10 years, the design of ALS epidemiological studies has evolved to focus on a prospective, population based methodology, employing the El E...
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description | Amyotrophic lateral sclerosis (ALS) is a relatively rare disease with a reported population incidence of between 1.5 and 2.5 per 100 000 per year. Over the past 10 years, the design of ALS epidemiological studies has evolved to focus on a prospective, population based methodology, employing the El Escorial criteria and multiple sources of data to ensure complete case ascertainment. Five such studies, based in Europe and North America, have been published and show remarkably consistent incidence figures among their respective Caucasian populations. Population based studies have been useful in defining clinical characteristics and prognostic indicators in ALS. However, many epidemiological questions remain that cannot be resolved by any of the existing population based datasets. The working hypotheses is that ALS, like other chronic diseases, is a complex genetic condition, and the relative contributions of individual environmental and genetic factors are likely to be relatively small. Larger studies are required to characterise risks and identify subpopulations that might be suitable for further study. This current paper outlines the contribution of the various population based registers, identifies the limitations of the existing datasets and proposes a mechanism to improve the future design and output of descriptive epidemiological studies. |
doi_str_mv | 10.1136/jnnp.2006.104828 |
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Over the past 10 years, the design of ALS epidemiological studies has evolved to focus on a prospective, population based methodology, employing the El Escorial criteria and multiple sources of data to ensure complete case ascertainment. Five such studies, based in Europe and North America, have been published and show remarkably consistent incidence figures among their respective Caucasian populations. Population based studies have been useful in defining clinical characteristics and prognostic indicators in ALS. However, many epidemiological questions remain that cannot be resolved by any of the existing population based datasets. The working hypotheses is that ALS, like other chronic diseases, is a complex genetic condition, and the relative contributions of individual environmental and genetic factors are likely to be relatively small. Larger studies are required to characterise risks and identify subpopulations that might be suitable for further study. This current paper outlines the contribution of the various population based registers, identifies the limitations of the existing datasets and proposes a mechanism to improve the future design and output of descriptive epidemiological studies.</description><identifier>ISSN: 0022-3050</identifier><identifier>EISSN: 1468-330X</identifier><identifier>DOI: 10.1136/jnnp.2006.104828</identifier><identifier>PMID: 18079297</identifier><identifier>CODEN: JNNPAU</identifier><language>eng</language><publisher>London: BMJ Publishing Group Ltd</publisher><subject>Adult ; Aged ; Alzheimer's disease ; Amyotrophic Lateral Sclerosis ; Amyotrophic Lateral Sclerosis - epidemiology ; Amyotrophic Lateral Sclerosis - mortality ; Amyotrophic Lateral Sclerosis - therapy ; Biological and medical sciences ; Clinical trials ; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases ; Dementia ; Dementia - epidemiology ; Epidemiology ; Female ; Genotype & phenotype ; Humans ; Incidence ; Interdisciplinary Communication ; Life Sciences ; Male ; Medical sciences ; Middle Aged ; Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis ; Neurology ; Palliative Care ; Parkinson Disease ; Population ; Prevalence ; Registries ; Risk Factors ; Santé publique et épidémiologie ; Sex Distribution ; Studies ; Survival Rate ; Time Factors</subject><ispartof>Journal of neurology, neurosurgery and psychiatry, 2008-01, Vol.79 (1), p.6-11</ispartof><rights>2008 BMJ Publishing Group</rights><rights>2008 INIST-CNRS</rights><rights>Copyright: 2008 2008 BMJ Publishing Group</rights><rights>Distributed under a Creative Commons Attribution 4.0 International License</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-b494t-b3f3eadb93003435bdafae0b5b8dc1b6c0c369e0cd1803d625d4fd17d8fa01513</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://jnnp.bmj.com/content/79/1/6.full.pdf$$EPDF$$P50$$Gbmj$$H</linktopdf><linktohtml>$$Uhttps://jnnp.bmj.com/content/79/1/6.full$$EHTML$$P50$$Gbmj$$H</linktohtml><link.rule.ids>114,115,230,314,780,784,885,3196,4024,23571,27923,27924,27925,77600,77631</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=19895644$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/18079297$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink><backlink>$$Uhttps://hal.science/hal-00337671$$DView record in HAL$$Hfree_for_read</backlink></links><search><creatorcontrib>Logroscino, G</creatorcontrib><creatorcontrib>Traynor, B J</creatorcontrib><creatorcontrib>Hardiman, O</creatorcontrib><creatorcontrib>Chio’, A</creatorcontrib><creatorcontrib>Couratier, P</creatorcontrib><creatorcontrib>Mitchell, J D</creatorcontrib><creatorcontrib>Swingler, R J</creatorcontrib><creatorcontrib>Beghi, E</creatorcontrib><creatorcontrib>EURALS</creatorcontrib><creatorcontrib>for EURALS</creatorcontrib><title>Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues</title><title>Journal of neurology, neurosurgery and psychiatry</title><addtitle>J Neurol Neurosurg Psychiatry</addtitle><description>Amyotrophic lateral sclerosis (ALS) is a relatively rare disease with a reported population incidence of between 1.5 and 2.5 per 100 000 per year. Over the past 10 years, the design of ALS epidemiological studies has evolved to focus on a prospective, population based methodology, employing the El Escorial criteria and multiple sources of data to ensure complete case ascertainment. Five such studies, based in Europe and North America, have been published and show remarkably consistent incidence figures among their respective Caucasian populations. Population based studies have been useful in defining clinical characteristics and prognostic indicators in ALS. However, many epidemiological questions remain that cannot be resolved by any of the existing population based datasets. The working hypotheses is that ALS, like other chronic diseases, is a complex genetic condition, and the relative contributions of individual environmental and genetic factors are likely to be relatively small. Larger studies are required to characterise risks and identify subpopulations that might be suitable for further study. This current paper outlines the contribution of the various population based registers, identifies the limitations of the existing datasets and proposes a mechanism to improve the future design and output of descriptive epidemiological studies.</description><subject>Adult</subject><subject>Aged</subject><subject>Alzheimer's disease</subject><subject>Amyotrophic Lateral Sclerosis</subject><subject>Amyotrophic Lateral Sclerosis - epidemiology</subject><subject>Amyotrophic Lateral Sclerosis - mortality</subject><subject>Amyotrophic Lateral Sclerosis - therapy</subject><subject>Biological and medical sciences</subject><subject>Clinical trials</subject><subject>Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases</subject><subject>Dementia</subject><subject>Dementia - epidemiology</subject><subject>Epidemiology</subject><subject>Female</subject><subject>Genotype & phenotype</subject><subject>Humans</subject><subject>Incidence</subject><subject>Interdisciplinary Communication</subject><subject>Life Sciences</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis</subject><subject>Neurology</subject><subject>Palliative Care</subject><subject>Parkinson Disease</subject><subject>Population</subject><subject>Prevalence</subject><subject>Registries</subject><subject>Risk Factors</subject><subject>Santé publique et épidémiologie</subject><subject>Sex Distribution</subject><subject>Studies</subject><subject>Survival Rate</subject><subject>Time Factors</subject><issn>0022-3050</issn><issn>1468-330X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2008</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><recordid>eNqF0c-L1DAUB_Aiijuu3j1JQBREOr78aNrsbRl1V5nVi4rgIaRJ6mZsm5q0o_Pfm9JhF7xsLoHk8x4v-WbZUwxrjCl_s-v7YU0A-BoDq0h1L1thxqucUvh-P1sBEJJTKOAkexTjDuZViYfZCa6gFESUq-zHWxt1cMPo9hbZwRnbOd_6nwfkG6S6gx-DH66dRq0abVAtirq1wUcXz1Bv_yC7TyW9tkj1Bk199O3eGuRinGx8nD1oVBvtk-N-mn19_-7L5jLffr74sDnf5jUTbMxr2lCrTC0oAGW0qI1qlIW6qCujcc01aMqFBW3S2NRwUhjWGFyaqlGAC0xPs1dL32vVyiG4ToWD9MrJy_OtnM9SX1ryEu9n-3KxQ_C_04yj7FzUtm1Vb_0UZQkYiBDkTkiAsZKyGT7_D-78FPr0YInLChNGBKdJwaJ0-rwYbHMzKAY5ZynnLOWcpVyyTCXPjo2nurPmtuAYXgIvjkBFrdomqF67eOtEJQrOWHL54lwc7d-bexV-SV7SspCfvm3k1XZTXRQfryRP_vXi625395j_AEnQxD8</recordid><startdate>200801</startdate><enddate>200801</enddate><creator>Logroscino, G</creator><creator>Traynor, B J</creator><creator>Hardiman, O</creator><creator>Chio’, A</creator><creator>Couratier, P</creator><creator>Mitchell, J D</creator><creator>Swingler, R J</creator><creator>Beghi, E</creator><general>BMJ Publishing Group Ltd</general><general>BMJ</general><general>BMJ Publishing Group LTD</general><general>BMJ Publishing Group</general><scope>BSCLL</scope><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7RV</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>88G</scope><scope>88I</scope><scope>8AF</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>BTHHO</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>KB0</scope><scope>M0S</scope><scope>M1P</scope><scope>M2M</scope><scope>M2P</scope><scope>NAPCQ</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>PSYQQ</scope><scope>Q9U</scope><scope>7TK</scope><scope>7X8</scope><scope>1XC</scope></search><sort><creationdate>200801</creationdate><title>Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues</title><author>Logroscino, G ; Traynor, B J ; Hardiman, O ; Chio’, A ; Couratier, P ; Mitchell, J D ; Swingler, R J ; Beghi, E</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-b494t-b3f3eadb93003435bdafae0b5b8dc1b6c0c369e0cd1803d625d4fd17d8fa01513</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2008</creationdate><topic>Adult</topic><topic>Aged</topic><topic>Alzheimer's disease</topic><topic>Amyotrophic Lateral Sclerosis</topic><topic>Amyotrophic Lateral Sclerosis - epidemiology</topic><topic>Amyotrophic Lateral Sclerosis - mortality</topic><topic>Amyotrophic Lateral Sclerosis - therapy</topic><topic>Biological and medical sciences</topic><topic>Clinical trials</topic><topic>Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases</topic><topic>Dementia</topic><topic>Dementia - epidemiology</topic><topic>Epidemiology</topic><topic>Female</topic><topic>Genotype & phenotype</topic><topic>Humans</topic><topic>Incidence</topic><topic>Interdisciplinary Communication</topic><topic>Life Sciences</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Middle Aged</topic><topic>Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis</topic><topic>Neurology</topic><topic>Palliative Care</topic><topic>Parkinson Disease</topic><topic>Population</topic><topic>Prevalence</topic><topic>Registries</topic><topic>Risk Factors</topic><topic>Santé publique et épidémiologie</topic><topic>Sex Distribution</topic><topic>Studies</topic><topic>Survival Rate</topic><topic>Time Factors</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Logroscino, G</creatorcontrib><creatorcontrib>Traynor, B J</creatorcontrib><creatorcontrib>Hardiman, O</creatorcontrib><creatorcontrib>Chio’, A</creatorcontrib><creatorcontrib>Couratier, P</creatorcontrib><creatorcontrib>Mitchell, J D</creatorcontrib><creatorcontrib>Swingler, R J</creatorcontrib><creatorcontrib>Beghi, E</creatorcontrib><creatorcontrib>EURALS</creatorcontrib><creatorcontrib>for EURALS</creatorcontrib><collection>Istex</collection><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Proquest Nursing & Allied Health Source</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>Psychology Database (Alumni)</collection><collection>Science Database (Alumni Edition)</collection><collection>STEM Database</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>ProQuest Central</collection><collection>BMJ Journals</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>SciTech Premium Collection</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Psychology Database</collection><collection>Science Database</collection><collection>Nursing & Allied Health Premium</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>ProQuest One Psychology</collection><collection>ProQuest Central Basic</collection><collection>Neurosciences Abstracts</collection><collection>MEDLINE - Academic</collection><collection>Hyper Article en Ligne (HAL)</collection><jtitle>Journal of neurology, neurosurgery and psychiatry</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Logroscino, G</au><au>Traynor, B J</au><au>Hardiman, O</au><au>Chio’, A</au><au>Couratier, P</au><au>Mitchell, J D</au><au>Swingler, R J</au><au>Beghi, E</au><aucorp>EURALS</aucorp><aucorp>for EURALS</aucorp><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues</atitle><jtitle>Journal of neurology, neurosurgery and psychiatry</jtitle><addtitle>J Neurol Neurosurg Psychiatry</addtitle><date>2008-01</date><risdate>2008</risdate><volume>79</volume><issue>1</issue><spage>6</spage><epage>11</epage><pages>6-11</pages><issn>0022-3050</issn><eissn>1468-330X</eissn><coden>JNNPAU</coden><abstract>Amyotrophic lateral sclerosis (ALS) is a relatively rare disease with a reported population incidence of between 1.5 and 2.5 per 100 000 per year. Over the past 10 years, the design of ALS epidemiological studies has evolved to focus on a prospective, population based methodology, employing the El Escorial criteria and multiple sources of data to ensure complete case ascertainment. Five such studies, based in Europe and North America, have been published and show remarkably consistent incidence figures among their respective Caucasian populations. Population based studies have been useful in defining clinical characteristics and prognostic indicators in ALS. However, many epidemiological questions remain that cannot be resolved by any of the existing population based datasets. The working hypotheses is that ALS, like other chronic diseases, is a complex genetic condition, and the relative contributions of individual environmental and genetic factors are likely to be relatively small. Larger studies are required to characterise risks and identify subpopulations that might be suitable for further study. This current paper outlines the contribution of the various population based registers, identifies the limitations of the existing datasets and proposes a mechanism to improve the future design and output of descriptive epidemiological studies.</abstract><cop>London</cop><pub>BMJ Publishing Group Ltd</pub><pmid>18079297</pmid><doi>10.1136/jnnp.2006.104828</doi><tpages>6</tpages></addata></record> |
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subjects | Adult Aged Alzheimer's disease Amyotrophic Lateral Sclerosis Amyotrophic Lateral Sclerosis - epidemiology Amyotrophic Lateral Sclerosis - mortality Amyotrophic Lateral Sclerosis - therapy Biological and medical sciences Clinical trials Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases Dementia Dementia - epidemiology Epidemiology Female Genotype & phenotype Humans Incidence Interdisciplinary Communication Life Sciences Male Medical sciences Middle Aged Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis Neurology Palliative Care Parkinson Disease Population Prevalence Registries Risk Factors Santé publique et épidémiologie Sex Distribution Studies Survival Rate Time Factors |
title | Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues |
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