Epidemiology of ALS in Italy
A 10-year prospective population-based study
Abstract
Objective: To describe the temporal patterns of incidence and demographic characteristics of amyotrophic lateral sclerosis (ALS) in Piemonte and Valle d’Aosta, Italy, in the 10-year period 1995 through 2004.
Methods: The Piemonte and Valle d’Aosta Register for ALS (PARALS) is a prospective register collecting all cases of ALS incident in two regions of northwestern Italy (population: 4,332,842) since 1995. The cases are identified using several concurrent sources. ALS diagnosis is based on El Escorial criteria.
Results: During the 10-year period of observation, 1,347 residents in the study area were diagnosed with ALS. In 1,260 of these cases, a diagnosis of definite or probable ALS was made at presentation or during the follow-up. The mean annual crude incidence rate was 2.90/100,000 population (95% confidence interval [CI], 2.72 to 3.09). The crude prevalence rate (December 31, 2004) was 7.89 (95% CI, 7.09 to 8.75)/100,000 population. According to the capture-recapture estimation, 27 patients were unobserved, thus increasing the annual observed crude incidence to 2.96/100,000 population. The incidence rate did not show any relevant variation during the 10-year period of the study and was constantly higher among men. The demographics and clinical features did not change between the 1995–1999 and the 2000–2004 cohorts, with the notable exception of the mean time delay from onset to diagnosis, which was significantly decreased in the last 5-year period.
Conclusions: In the examined decade, the incidence of amyotrophic lateral sclerosis in Italy has been stable, and the clinical and demographic characteristics of the patients have shown no relevant modifications.
ALS = amyotrophic lateral sclerosis; CI = confidence interval; EEC = El Escorial diagnostic criteria; EEC-R = El Escorial revised criteria; MND = motor neuron disease; PARALS = Piemonte and Valle d’Aosta Register for ALS; PCRA = Piemonte Central Regional Archive; VACRA = Valle d’Aosta Central Regional Archive.
Get full access to this article
View all available purchase options and get full access to this article.
Supplementary Material
REFERENCES
1.
Chiò A, Traynor BJ, Lombardo F, et al. Prevalence of SOD1 mutations in the Italian ALS population. Neurology 2008;70:533–537.
2.
McGuire V, Longstreth WT Jr, Koepsell TD, van Belle G. Incidence of amyotrophic lateral sclerosis in three counties in western Washington state. Neurology 1996;47:571–573.
3.
Piemonte and Valle d’Aosta Register for Amyotrophic Lateral Sclerosis (PARALS). Incidence of ALS in Italy: evidence for a uniform frequency in Western countries. Neurology 2001;56:239–244.
4.
Logroscino G, Beghi E, Zoccolella S, et al. Incidence of amyotrophic lateral sclerosis in southern Italy: a population-based study. J Neurol Neurosurg Psychiatry 2005;76:1094–1098.
5.
Forbes RB, Colville S, Parratt J, Swingler RJ. The incidence of motor neuron disease in Scotland. J Neurol 2007;254:866–869.
6.
O’Toole O, Traynor BJ, Brennan P, et al. Epidemiology and clinical features of amyotrophic lateral sclerosis in Ireland between 1995 and 2004. J Neurol Neurosurg Psychiatry 2007;79:30–32.
7.
Beghi E, Millul A, Micheli A, Vitelli E, Logroscino G, for the SLALOM Group. Incidence of ALS in Lombardy, Italy. Neurology 2007;68:141–145.
8.
Seljeseth YM, Vollset SE, Tysnes OB. Increasing mortality from amyotrophic lateral sclerosis in Norway? Neurology 2000;55:1262–1266.
9.
Noonan CW, White MC, Thurman D, Wong LY. Temporal and geographic variation in United States motor neuron disease mortality, 1969 to 1998. Neurology 2005;64:1215–1221.
10.
Sejvar JJ, Holman RC, Bresee JS, Kochanek KD, Schonberger LB. Amyotrophic lateral sclerosis mortality in the United States, 1979–2001. Neuroepidemiology 2005;25:144–152.
11.
Messina R, ed. Sanità in Cifre. Calderini: Ozzano dell’Emilia (BO);
12.
Brooks BR, Miller RG, Swash M, Munsat TL, World Federation of Neurology Research Group on Motor Neuron Diseases. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 2000;1:293–299.
13.
Brooks BR. El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis: Subcommittee on Motor Neuron Diseases/Amyotrophic Lateral Sclerosis of the World Federation of Neurology Research Group on Neuromuscular Diseases and the El Escorial Clinical Limits of Amyotrophic Lateral Sclerosis workshop contributors. J Neurol Sci 1994;124(suppl):96–107.
14.
Schoemberg BS. Calculating confidence intervals for rates and ratios. Neuroepidemiology 1983;2:257–265.
15.
Hook E, Regal R. Capture recapture methods in epidemiology: methods and limitations. Epidemiol Rev 1995;17:243–264.
16.
Sorenson EJ, Stalker AP, Kurland LT, Windebank AJ. Amyotrophic lateral sclerosis in Olmsted County, Minnesota, 1925 to 1998. Neurology 2002;59:280–282.
17.
Traynor BJ, Codd MB, Corr B, Forde C, Frost E, Hardiman O. Amyotrophic lateral sclerosis mimic syndromes: a population-based study. Arch Neurol 2000;57:109–113.
18.
Davenport RJ, Swingler RJ, Chancellor AM, et al. Avoiding false positive diagnoses of motor neuron disease: lessons from the Scottish Motor Neuron Disease Register. J Neurol Neurosurg Psychiatry 1996;60:147–151.
19.
Forbes RB, Colville S, Swingler RJ. Scottish ALS/MND Register: the epidemiology of amyotrophic lateral sclerosis (ALS/MND) in people aged 80 or over. Age Ageing 2004;33:131–134.
20.
Qiu C, De Ronchi D, Fratiglioni L. The epidemiology of the dementias: an update. Curr Opin Psychiatry 2007;20:380–385.
21.
Twelves D, Perkins KS, Counsell C. Systematic review of incidence studies of Parkinson’s disease. Mov Disord 2003;18:19–31.
22.
Johnston CA, Stanton BA, Turner MR, et al. Amyotrophic lateral sclerosis in an urban setting: a population-based study of inner city London. J Neurol 2006;253:1642–1643.
23.
Logroscino G, Traynor BJ, Hardiman O, et al. Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues. J Neurol Neurosurg Psychiatry 2008;79:6–11.
24.
Beghi E, Mennini T, Bendotti C, et al. The heterogeneity of amyotrophic lateral sclerosis: a possible explanation of treatment failure. Curr Med Chem 2007;14:3185–3200.
Information & Authors
Information
Published In
Copyright
© 2009.
Publication History
Published online: February 23, 2009
Published in print: February 24, 2009
Notes
Authors
Metrics & Citations
Metrics
Citation information is sourced from Crossref Cited-by service.
Citations
Download Citations
If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. Select your manager software from the list below and click Download.
Cited By
- Incidence of amyotrophic lateral sclerosis before and during the COVID-19 pandemic: evidence from an 8-year population-based study in Central Italy based on healthcare utilization databases, Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 25, 5-6, (554-562), (2024).https://doi.org/10.1080/21678421.2024.2336127
- Incidence of amyotrophic lateral sclerosis in Chile, Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 25, 5-6, (528-532), (2024).https://doi.org/10.1080/21678421.2024.2329706
- Brain–body mechanisms contribute to sexual dimorphism in amyotrophic lateral sclerosis, Nature Reviews Neurology, 20, 8, (475-494), (2024).https://doi.org/10.1038/s41582-024-00991-7
- Pan-neuronal expression of human mutant SOD1 in Drosophila impairs survival and motor performance, induces early neuroinflammation and chromosome aberrations, Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, 1870, 5, (167192), (2024).https://doi.org/10.1016/j.bbadis.2024.167192
- The BciAi4SLA Project: Towards a User-Centered BCI, Electronics, 12, 5, (1234), (2023).https://doi.org/10.3390/electronics12051234
- Estimated Familial Amyotrophic Lateral Sclerosis Proportion, Neurology Genetics, 9, 6, (2023)./doi/10.1212/NXG.0000000000200109
- ALS-linked FUS R521C disrupts arginine methylation of UBAP2L and stress granule dynamics, Journal of Analytical Science and Technology, 14, 1, (2023).https://doi.org/10.1186/s40543-023-00389-y
- LAENALS: epidemiological and clinical features of amyotrophic lateral sclerosis in Latin America, Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 25, 1-2, (119-127), (2023).https://doi.org/10.1080/21678421.2023.2271517
- Clinical epidemiology of amyotrophic lateral sclerosis in Liguria, Italy: a ten year follow up study, Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 25, 1-2, (104-111), (2023).https://doi.org/10.1080/21678421.2023.2260842
- Epidemiological and clinical profile of amyotrophic lateral sclerosis in Ethiopia: a 5-year multicenter retrospective study, Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 24, 7-8, (678-686), (2023).https://doi.org/10.1080/21678421.2023.2238016
- See more
Loading...
View Options
Login options
Check if you have access through your login credentials or your institution to get full access on this article.
Personal login Institutional LoginPurchase Options
The neurology.org payment platform is currently offline. Our technical team is working as quickly as possible to restore service.
If you need immediate support or to place an order, please call or email customer service:
- 1-800-638-3030 for U.S. customers - 8:30 - 7 pm ET (M-F)
- 1-301-223-2300 for customers outside the U.S. - 8:30 - 7 pm ET (M-F)
- [email protected]
We appreciate your patience during this time and apologize for any inconvenience.