Rate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis.
AffiliationDeparment of Neurology, Beaumont Hospital, Beaumont Rd, Dublin 9, Ireland., email@example.com
MeSHAmyotrophic Lateral Sclerosis/*epidemiology/genetics
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CitationJ Neurol Neurosurg Psychiatry. 2011 Jun;82(6):623-7. Epub 2010 Nov 3.
JournalJournal of neurology, neurosurgery, and psychiatry
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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