Clinical epidemiology of ALS in Liguria, Italy

Abstract

Our objective was to assess the incidence and trends of amyotrophic lateral sclerosis (ALS) in Liguria, a north-west region of Italy, utilizing a prospective design. Liguria (1,615,064 residents in 2010) is the site of a multicentre-multisource prospective population based registry called LIGALS (Liguria Amyotrophic Lateral Sclerosis Registry). All incident ALS cases during the period 2009–2010 were enrolled and followed up. Cases were identified using several concurrent sources. ALS diagnosis was based on the revised El Escorial criteria. One hundred and four cases were enrolled, generating an annual crude incidence of 3.22/100,000 (95% CI 2.66−3.90), with a male/female ratio of 1.34. The annual standardized incidence, age and gender adjusted to the 2001 Italian population, was 2.51. At last observation on 1 March 2012, 45% of patients registered in the LIGALS had died, with a median survival of 45 months from symptoms onset. According to capture-recapture estimation, three patients were unobserved. For both genders, demographic and clinical features were collected. In conclusion, comparing these data to those of epidemiological studies with a similar prospective design, the occurrence of ALS is similar. The observed crude incidence was higher compared to other Italian studies, due in part to a very careful case ascertainment and in part to a high percentage of the elderly in Liguria.

Key words::

• Epidemiology
• amyotrophic lateral sclerosis
• population based study
• incidence
• motor neuron diseases

Acknowledgements

This work has been possible thanks to:

• LIGALS members: Alberti Francesco (neurologist), Assini Andrea (neurologist), Balletto Nadia (palliative care specialist), Bandini Fabio (neurologist), Benedetti Luana (neurologist), Beronio Alessandro (neurologist), Cellotto Nadia (rehabilitation physician), DeMichelis Claudio (pulmonologist), DeMontis Stefania (nutritionist), Marogna Maura (neurologist), Mascolo Maria (neurologist), Monti Bragadin Margherita (rehabilitation physician), Narciso Eleonora (neurologist), Nicolini Antonello (pulmonologist), Pizio Nicola (neurologist), Pizzorno Matteo (neurologist), Rao Fabrizio (pulmonologist), Tassinari Tiziana (neurologist), Zatt Carla (neurologist), Zuccarino Riccardo (rehabilitation physician).

• ALS neurologists practising in region adjacent to the study area: Calvo Andrea, Chiò Adriano, Siciliano Gabriele.

• AISLA section of Genoa and section of Savona, Gigi Ghirotti Association.

• Rossetti Niccolò for technical support.

Declaration of interest: The authors report no conflict of interest. The authors alone are responsible for the content and writing of the article. This work was partly supported by grant ‘La Sclerosi Laterale Amiotrofica (SLA) e le Gravi Malattie del Motoneurone: dai Meccanismi Molecolari alla Presa in Carico del Paziente’, Regione Liguria to GLM and PM.