The DOI matches its Crossref record; 71 of the 71 references it cites were found in a registry or an index.
Rate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis
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.
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Cites
Genes mirror geography within Europe
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The natural history of amyotrophic lateral sclerosis
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Cited by
van Es, M. A., Hardiman, O., Chio, A., Al-Chalabi, A., Pasterkamp, R. J., Veldink, J. H., & van den Berg, L. H. (2017) · The Lancet · doi:10.1016/s0140-6736(17)31287-4 · Cited by 1,298
Self-citationThe epidemiology of ALS: a conspiracy of genes, environment and time
Al-Chalabi, A., & Hardiman, O. (2013) · Nature Reviews Neurology · doi:10.1038/nrneurol.2013.203 · Cited by 894
Self-citationA comprehensive review of amyotrophic lateral sclerosis
Zarei, S., Carr, K., Reiley, L., Diaz, K., Guerra, O., Altamirano, P., Pagani, W., Lodin, D., Orozco, G., & Chinea, A. (2015) · Surgical Neurology International · doi:10.4103/2152-7806.169561 · Cited by 709
Clinical genetics of amyotrophic lateral sclerosis: what do we really know?
Andersen, P. M., & Al-Chalabi, A. (2011) · Nature Reviews Neurology · doi:10.1038/nrneurol.2011.150 · Cited by 687
Clinical diagnosis and management of amyotrophic lateral sclerosis
Hardiman, O., van den Berg, L. H., & Kiernan, M. C. (2011) · Nature Reviews Neurology · doi:10.1038/nrneurol.2011.153 · Cited by 621
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Metadata
- Authors
- Byrne, S., Walsh, C., Lynch, C., Bede, P., Elamin, M., Kenna, K., McLaughlin, R., & Hardiman, O.
- Venue
- Journal of Neurology, Neurosurgery & Psychiatry
- Publisher
- BMJ
- Published
- 2010-11-03
- Volume
- 82
- Issue
- 6
- Pages
- 623-627
- ISSN
- 0022-3050 (ISSN-L)
- OpenAlex
- W2115450084
- Cited by
- 351
- References
- 71
- FWCI
- 2.55
- Type
- journal article
- Language
- en
- Open access
- closed
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