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Rate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis.

dc.contributor.authorByrne, Susan
dc.contributor.authorWalsh, Cathal
dc.contributor.authorLynch, Catherine
dc.contributor.authorBede, Peter
dc.contributor.authorElamin, Marwa
dc.contributor.authorKenna, Kevin
dc.contributor.authorMcLaughlin, Russell
dc.contributor.authorHardiman, Orla
dc.date.accessioned2012-02-01T10:02:15Z
dc.date.available2012-02-01T10:02:15Z
dc.date.issued2012-02-01T10:02:15Z
dc.identifier.citationJ Neurol Neurosurg Psychiatry. 2011 Jun;82(6):623-7. Epub 2010 Nov 3.en_GB
dc.identifier.issn1468-330X (Electronic)en_GB
dc.identifier.issn0022-3050 (Linking)en_GB
dc.identifier.pmid21047878en_GB
dc.identifier.doi10.1136/jnnp.2010.224501en_GB
dc.identifier.urihttp://hdl.handle.net/10147/207219
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.
dc.language.isoengen_GB
dc.subject.meshAmyotrophic Lateral Sclerosis/*epidemiology/geneticsen_GB
dc.subject.meshHumansen_GB
dc.subject.meshMutationen_GB
dc.subject.meshSuperoxide Dismutase/geneticsen_GB
dc.titleRate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis.en_GB
dc.contributor.departmentDeparment of Neurology, Beaumont Hospital, Beaumont Rd, Dublin 9, Ireland., suabyrne@gmail.comen_GB
dc.identifier.journalJournal of neurology, neurosurgery, and psychiatryen_GB
dc.description.provinceLeinster
html.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.


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