Incidence of amyotrophic lateral sclerosis in Sicily: A population based study

Abstract

Our objective was to investigate incidence of amyotrophic lateral sclerosis (ALS) in Sicily, southern Italy, by means of a population based study. We included people with ALS resident in five Sicilian provinces, whose onset occurred in the two-year period 2005−2006 (population at 31 December 2006: 3,481,096 inhabitants). A multisource case-finding procedure was adopted and patients were classified as affected by ALS according to revised El Escorial criteria. During the two-year surveillance period, 97 patients meeting eligibility criteria included 57 males (58.8%) and 40 females (41.2%). Crude annual incidence rate was 1.4/100,000 person years (95% CI 1.33−1.47). The incidence rate was higher in males (1.71/100,000; CI 1.61−1.81) than in females (1.11/100,000; CI 1.01−1.21). Standardized incidence rate for the total population in the 45−74-years-old age group was 3.22 (CI 3.11−3.33). Prevalence rate was 6.0/100,000 (CI 5.97−6.03), higher in males (7.1/100,000; CI 7.02−7.18) than females (4.9/100,000; CI 4.86−4.94). In conclusion, ALS rates observed in the present study are higher in males than females, with a peak of incidence at 70 years of age in both genders. These findings are consistent with those of other population based European studies.

Key words::

• Amyotrophic lateral sclerosis
• epidemiology
• incidence
• population based
• Sicily

Acknowledgements

The authors thank Mario Zappia and Giovanni Majorana for their helpful collaboration. The authors also thank the anonymous referees for their contribution to a substantial revision and improvement of the study.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.