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dc.contributor.authorWALSH, CATHALen
dc.contributor.authorBEDE, PETERen
dc.contributor.authorHARDIMAN, ORLAen
dc.contributor.authorMCLAUGHLIN, RUSSELLen
dc.contributor.authorWALTON, CLAIRen
dc.date.accessioned2011-03-16T16:18:37Z
dc.date.available2011-03-16T16:18:37Z
dc.date.issued2011en
dc.date.submitted2011en
dc.identifier.citationByrne S, Walsh C, Lynch C, Bede P, Elamin M, Kenna K, McLaughlin R, Hardiman O, Rate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis., Journal of neurology, neurosurgery, and psychiatry, 82, 6, 2011, 623 - 627en
dc.identifier.issn0022-3050en
dc.identifier.otherYen
dc.descriptionPUBLISHEDen
dc.description.abstractBackground The population rate of familial amyotrophic lateral sclerosis (FALS) is frequently reported as 10%. However, a systematic review and meta-analysis of the true population based frequency of FALS has never been performed. Method A Medline literature review identified all original articles reporting a rate of FALS. Studies were grouped according to the type of data presented and examined for sources of case ascertainment. A systematic review and meta-analysis of reported rates of FALS was then conducted to facilitate comparison between studies and calculate a pooled rate of FALS. Results 38 papers reported a rate of FALS. Thirty-three papers were included in analysis and the rate of FALS for all studies was 4.6% (95% CI 3.9% to 5.5%). Restricting the analysis to prospective population based registry data revealed a rate of 5.1% (95% CI 4.1% to 6.1%). The incidence of FALS was lower in southern Europe. There was no correlation between rate of FALS and reported SOD1 mutation rates. Conclusion The rate of FALS among prospective population based registries is 5.1% (CI 4.1 to 6.1%), and not 10% as is often stated. Further detailed prospective population based studies of familial ALS are required to confirm this rate.en
dc.description.sponsorshipThis work was supported by the IMNDA, IMNDRF and HRB funding.en
dc.format.extent623en
dc.format.extent627en
dc.language.isoenen
dc.relation.ispartofseriesJournal of neurology, neurosurgery, and psychiatryen
dc.relation.ispartofseries82en
dc.relation.ispartofseries6en
dc.rightsYen
dc.subjectClinical Medicineen
dc.subjectapplied statisticsen
dc.subjectfamilial amyotrophic lateral sclerosisen
dc.titleRate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis.en
dc.typeJournal Articleen
dc.type.supercollectionscholarly_publicationsen
dc.type.supercollectionrefereed_publicationsen
dc.identifier.peoplefinderurlhttp://people.tcd.ie/walshcen
dc.identifier.peoplefinderurlhttp://people.tcd.ie/pbedeen
dc.identifier.peoplefinderurlhttp://people.tcd.ie/hardimaoen
dc.identifier.peoplefinderurlhttp://people.tcd.ie/cwaltonen
dc.identifier.rssinternalid69955en
dc.identifier.doihttp://dx.doi.org/10.1136/jnnp.2010.224501en
dc.subject.TCDThemeNeuroscienceen
dc.identifier.rssurihttp://dx.doi.org/10.1136/jnnp.2010.224501en
dc.contributor.sponsorHealth Research Board (HRB)en
dc.identifier.urihttp://hdl.handle.net/2262/53330


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