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.

Amyotrophic Lateral Sclerosis Descriptive Studies: Not Only Rates and Frequencies

“…An incidence rate of 1.4/100,000 was reported both in Catalonia [11] and in Sicily [12] similar to what has been reported previously in Apulia (1.7/100,000). Overall, the incidence in these southern registries is about half of what has been reported in Friuli and Piedmont.…”

Amyotrophic lateral sclerosis surveillance in Baltimore and Philadelphia

“…18 The results we found regarding the time between symptom onset and diagnosis also agree with previous research. 14,16 …”

“…11–18 The median duration of time from symptom onset to diagnosis has been reported to range from 10 to 36 months. 14,16 Much of the published epidemiological data regarding ALS are from outside of the United States (U.S.), and case ascertainment methodologies vary to include the use of existing data sets, neurologist reports, death certificates, hospital records, and pharmacological records. 11 …”

Epidemiology of Amyotrophic Lateral Sclerosis and Effect of Riluzole on Disease Course

“…The proportion of unobserved cases in epidemiological studies on ALS has previously been estimated to be as high as 28% [14,20]. Not accounting for such an important bias is therefore likely to lead to a substantial underestimation of the true IR and PR [10,21].…”