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 Volume 72, Number 8, February 24, 2009
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Right arrow Amyotrophic lateral sclerosis
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NEUROLOGY 2009;72:725-731
© 2009 American Academy of Neurology

Epidemiology of ALS in Italy

A 10-year prospective population-based study A. Chiò, MD, G. Mora, MD, A. Calvo, MD, L. Mazzini, MD, E. Bottacchi, MD, R. Mutani, MD 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.

Address correspondence and reprint requests to Prof. Adriano Chiò, Department of Neuroscience, via Cherasco 15, 10126 Torino, Italy achio{at}usa.net

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.

Supplemental data at www.neurology.org

*See the appendix for a list of participating centers.

Supported by Regione Piemonte (Ricerca Finalizzata 2002, grant 12944; Ricerca Scientifica Applicata 2004, grant A317), and by Compagnia di San Paolo (grant 2003.0078).

Disclosure: The authors report no disclosures.

Received August 4, 2008. Accepted in final form November 24, 2008.