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

Hdl Handle:
http://hdl.handle.net/10147/207219
Title:
Rate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis.
Authors:
Byrne, Susan; Walsh, Cathal; Lynch, Catherine; Bede, Peter; Elamin, Marwa; Kenna, Kevin; McLaughlin, Russell; Hardiman, Orla
Affiliation:
Deparment of Neurology, Beaumont Hospital, Beaumont Rd, Dublin 9, Ireland., suabyrne@gmail.com
Citation:
J Neurol Neurosurg Psychiatry. 2011 Jun;82(6):623-7. Epub 2010 Nov 3.
Journal:
Journal of neurology, neurosurgery, and psychiatry
Issue Date:
1-Feb-2012
URI:
http://hdl.handle.net/10147/207219
DOI:
10.1136/jnnp.2010.224501
PubMed ID:
21047878
Abstract:
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.
Language:
eng
MeSH:
Amyotrophic Lateral Sclerosis/*epidemiology/genetics; Humans; Mutation; Superoxide Dismutase/genetics
ISSN:
1468-330X (Electronic); 0022-3050 (Linking)

Full metadata record

DC FieldValue Language
dc.contributor.authorByrne, Susanen_GB
dc.contributor.authorWalsh, Cathalen_GB
dc.contributor.authorLynch, Catherineen_GB
dc.contributor.authorBede, Peteren_GB
dc.contributor.authorElamin, Marwaen_GB
dc.contributor.authorKenna, Kevinen_GB
dc.contributor.authorMcLaughlin, Russellen_GB
dc.contributor.authorHardiman, Orlaen_GB
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.en_GB
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-

Related articles on PubMed

All Items in Lenus, The Irish Health Repository are protected by copyright, with all rights reserved, unless otherwise indicated.