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February 23, 2009

Epidemiology of ALS in Italy
A 10-year prospective population-based study

February 24, 2009 issue
72 (8) 725-731

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.

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Information & Authors

Information

Published In

Neurology®
Volume 72Number 8February 24, 2009
Pages: 725-731
PubMed: 19237701

Publication History

Published online: February 23, 2009
Published in print: February 24, 2009

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Authors

Affiliations & Disclosures

A. Chiò, MD
From the Department of Neuroscience (A. Chiò, A. Calvo, R.M.), University of Torino; the Department of Neurological Rehabilitation (G.M.), Fondazione S. Maugeri, Clinica del Lavoro e della Riabilitazione, IRCCS, Scientific Institute of Pavia; the Department of Neurology (L.M.), University of Piemonte Orientale “Amedeo Avogadro,” Azienda Ospedaliera Maggiore, Novara; and the Department of Neurology (E.B.), Ospedale Regionale di Aosta, Italy.
G. Mora, MD
From the Department of Neuroscience (A. Chiò, A. Calvo, R.M.), University of Torino; the Department of Neurological Rehabilitation (G.M.), Fondazione S. Maugeri, Clinica del Lavoro e della Riabilitazione, IRCCS, Scientific Institute of Pavia; the Department of Neurology (L.M.), University of Piemonte Orientale “Amedeo Avogadro,” Azienda Ospedaliera Maggiore, Novara; and the Department of Neurology (E.B.), Ospedale Regionale di Aosta, Italy.
A. Calvo, MD
From the Department of Neuroscience (A. Chiò, A. Calvo, R.M.), University of Torino; the Department of Neurological Rehabilitation (G.M.), Fondazione S. Maugeri, Clinica del Lavoro e della Riabilitazione, IRCCS, Scientific Institute of Pavia; the Department of Neurology (L.M.), University of Piemonte Orientale “Amedeo Avogadro,” Azienda Ospedaliera Maggiore, Novara; and the Department of Neurology (E.B.), Ospedale Regionale di Aosta, Italy.
L. Mazzini, MD
From the Department of Neuroscience (A. Chiò, A. Calvo, R.M.), University of Torino; the Department of Neurological Rehabilitation (G.M.), Fondazione S. Maugeri, Clinica del Lavoro e della Riabilitazione, IRCCS, Scientific Institute of Pavia; the Department of Neurology (L.M.), University of Piemonte Orientale “Amedeo Avogadro,” Azienda Ospedaliera Maggiore, Novara; and the Department of Neurology (E.B.), Ospedale Regionale di Aosta, Italy.
E. Bottacchi, MD
From the Department of Neuroscience (A. Chiò, A. Calvo, R.M.), University of Torino; the Department of Neurological Rehabilitation (G.M.), Fondazione S. Maugeri, Clinica del Lavoro e della Riabilitazione, IRCCS, Scientific Institute of Pavia; the Department of Neurology (L.M.), University of Piemonte Orientale “Amedeo Avogadro,” Azienda Ospedaliera Maggiore, Novara; and the Department of Neurology (E.B.), Ospedale Regionale di Aosta, Italy.
R. Mutani, MD
From the Department of Neuroscience (A. Chiò, A. Calvo, R.M.), University of Torino; the Department of Neurological Rehabilitation (G.M.), Fondazione S. Maugeri, Clinica del Lavoro e della Riabilitazione, IRCCS, Scientific Institute of Pavia; the Department of Neurology (L.M.), University of Piemonte Orientale “Amedeo Avogadro,” Azienda Ospedaliera Maggiore, Novara; and the Department of Neurology (E.B.), Ospedale Regionale di Aosta, Italy.
On behalf of the PARALS
From the Department of Neuroscience (A. Chiò, A. Calvo, R.M.), University of Torino; the Department of Neurological Rehabilitation (G.M.), Fondazione S. Maugeri, Clinica del Lavoro e della Riabilitazione, IRCCS, Scientific Institute of Pavia; the Department of Neurology (L.M.), University of Piemonte Orientale “Amedeo Avogadro,” Azienda Ospedaliera Maggiore, Novara; and the Department of Neurology (E.B.), Ospedale Regionale di Aosta, Italy.

Notes

Address correspondence and reprint requests to Prof. Adriano Chiò, Department of Neuroscience, via Cherasco 15, 10126 Torino, Italy [email protected]

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