INCIDENCE OF AMYOTROPHIC LATERAL SCLEROSIS IN SICILY: A POPULATION BASED STUDY

Our objective was to investigate incidence of amyotrophic lateral sclerosis (ALS) in Sicily, southern Italy, by means of apopulation based study. We included people with ALS resident in fi ve Sicilian provinces, whose onset occurred in thetwo-year period 2005 2006 (population at 31 December 2006: 3,481,096 inhabitants). A multisource case-fi nding procedurewas adopted and patients were classifi ed as affected by ALS according to revised El Escorial criteria. During thetwo-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 totalpopulation 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 observedin the present study are higher in males than females, with a peak of incidence at 70 years of age in both genders. Thesefi ndings are consistent with those of other population based European studies