REVIEWS

Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues

G Logroscino¹, B J Traynor^2,3, O Hardiman⁴, A Chio’⁵, P Couratier⁶, J D Mitchell⁷, R J Swingler⁸, E Beghi⁹, for EURALS

¹ Department of Epidemiology HSPH, Harvard University, Boston, Massachusetts, USA
² Section on Developmental Genetic Epidemiology, National Institutes of Health, Bethesda, Maryland, USA
³ Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA
⁴ Department of Neurology, Beaumont Hospital and Royal College of Surgeons in Ireland, Dublin, Ireland
⁵ Dipartimento di Neuroscienze, Universitè di Torino, Torino, Italy
⁶ Service de Neurologie, CHU Limoges, Limoges, France
⁷ ALS Care and Research Centre, Royal Preston Hospital, Preston, UK
⁸ Department of Neurology, Ninewells Hospital, Dundee, UK
⁹ Istituto Ricerche Farmacologiche Mario Negri Milano and Clinica Neurologica, Universitè di Milano-Bicocca, Monza, Italy

Correspondence to:
Dr G Logroscino, Department of Epidemiology HSPH 3-819 Harvard University, 677 Huntington Avenue, Boston, Massachusetts 02115, USA; glogrosc{at}hsph.harvard.edu

Amyotrophic lateral sclerosis (ALS) is a relatively rare disease with a reported population incidence of between 1.5 and 2.5 per 100 000 per year. Over the past 10 years, the design of ALS epidemiological studies has evolved to focus on a prospective, population based methodology, employing the El Escorial criteria and multiple sources of data to ensure complete case ascertainment. Five such studies, based in Europe and North America, have been published and show remarkably consistent incidence figures among their respective Caucasian populations. Population based studies have been useful in defining clinical characteristics and prognostic indicators in ALS. However, many epidemiological questions remain that cannot be resolved by any of the existing population based datasets. The working hypotheses is that ALS, like other chronic diseases, is a complex genetic condition, and the relative contributions of individual environmental and genetic factors are likely to be relatively small. Larger studies are required to characterise risks and identify subpopulations that might be suitable for further study. This current paper outlines the contribution of the various population based registers, identifies the limitations of the existing datasets and proposes a mechanism to improve the future design and output of descriptive epidemiological studies.

CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

• Pesiridis, G. S., Lee, V. M.-Y., Trojanowski, J. Q. (2009). Mutations in TDP-43 link glycine-rich domain functions to amyotrophic lateral sclerosis. Hum Mol Genet 18: R156-R162 [Abstract] [Full Text]  
• Murray, K, Davenport, R J (2009). A rapidly progressive neuropathy. PN 9: 172-175 [Full Text]  
• Zaldivar, T., Gutierrez, J., Lara, G., Carbonara, M., Logroscino, G., Hardiman, O. (2009). Reduced frequency of ALS in an ethnically mixed population: A population-based mortality study. Neurology 72: 1640-1645 [Abstract] [Full Text]  
• Weisskopf, M G, Morozova, N, O'Reilly, E J, McCullough, M L, Calle, E E, Thun, M J, Ascherio, A (2009). Prospective study of chemical exposures and amyotrophic lateral sclerosis. J. Neurol. Neurosurg. Psychiatry 80: 558-561 [Abstract] [Full Text]  
• Wijesekera, L. C., Mathers, S., Talman, P., Galtrey, C., Parkinson, M. H., Ganesalingam, J., Willey, E., Ampong, M. A., Ellis, C. M., Shaw, C. E., Al-Chalabi, A., Leigh, P. N. (2009). Natural history and clinical features of the flail arm and flail leg ALS variants. Neurology 72: 1087-1094 [Abstract] [Full Text]  
• Chio, A., Mora, G., Calvo, A., Mazzini, L., Bottacchi, E., Mutani, R., On behalf of the PARALS, (2009). Epidemiology of ALS in Italy: A 10-year prospective population-based study. Neurology 72: 725-731 [Abstract] [Full Text]  
• Murphy, M., Quinn, S., Young, J., Parkin, P., Taylor, B. (2008). Increasing incidence of ALS in Canterbury, New Zealand: A 22-year study. Neurology 71: 1889-1895 [Abstract] [Full Text]  
• O'Toole, O, Traynor, B J, Brennan, P, Sheehan, C, Frost, E, Corr, B, Hardiman, O (2008). Epidemiology and clinical features of amyotrophic lateral sclerosis in Ireland between 1995 and 2004. J. Neurol. Neurosurg. Psychiatry 79: 30-32 [Abstract] [Full Text]  

eLetters:

Read all eLetters

Pathogenesis of ALS

Heikki Savolainen

JNNP Online, 9 Jan 2008 [Full text]