Rate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis.
Authors
Byrne, SusanWalsh, Cathal
Lynch, Catherine
Bede, Peter
Elamin, Marwa
Kenna, Kevin
McLaughlin, Russell
Hardiman, Orla
Affiliation
Deparment of Neurology, Beaumont Hospital, Dublin, Ireland.Issue Date
2010-11-03
Metadata
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Rate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis. 2010: J. Neurol. Neurosurg. Psychiatr.Journal
Journal of neurology, neurosurgery, and psychiatryDOI
10.1136/jnnp.2010.224501PubMed ID
21047878Abstract
Background 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.Item Type
Article In PressISSN
1468-330Xae974a485f413a2113503eed53cd6c53
10.1136/jnnp.2010.224501
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