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

Hdl Handle:
http://hdl.handle.net/10147/126131
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, Dublin, Ireland.
Citation:
Rate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis. 2010: J. Neurol. Neurosurg. Psychiatr.
Journal:
Journal of neurology, neurosurgery, and psychiatry
Issue Date:
3-Nov-2010
URI:
http://hdl.handle.net/10147/126131
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.
Item Type:
Article In Press
ISSN:
1468-330X

Full metadata record

DC FieldValue Language
dc.contributor.authorByrne, Susanen
dc.contributor.authorWalsh, Cathalen
dc.contributor.authorLynch, Catherineen
dc.contributor.authorBede, Peteren
dc.contributor.authorElamin, Marwaen
dc.contributor.authorKenna, Kevinen
dc.contributor.authorMcLaughlin, Russellen
dc.contributor.authorHardiman, Orlaen
dc.date.accessioned2011-03-29T14:48:59Z-
dc.date.available2011-03-29T14:48:59Z-
dc.date.issued2010-11-03-
dc.identifier.citationRate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis. 2010: J. Neurol. Neurosurg. Psychiatr.en
dc.identifier.issn1468-330X-
dc.identifier.pmid21047878-
dc.identifier.doi10.1136/jnnp.2010.224501-
dc.identifier.urihttp://hdl.handle.net/10147/126131-
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.languageENG-
dc.titleRate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis.en
dc.typeArticle In Pressen
dc.contributor.departmentDeparment of Neurology, Beaumont Hospital, Dublin, Ireland.en
dc.identifier.journalJournal of neurology, neurosurgery, and psychiatryen
dc.description.provinceLeinster-

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