Epidemiology

There are three forms of ALS, the most common, sporadic ALS (sALS); familial ALS (fALS), which is genetically present in only 149 patients from 53 families worldwide (including a rare juvenile form); and an endemic focus of ALS in the western Pacific.1 The worldwide annual incidence rate for sALS is 0.6 to 2.0 per 100,000, and the prevalence is 4 to 6 per 100,000.2 The mean age of onset for sALS is 56 years, with no gender differences noted. The incidence of fALS is only 10 percent of that of sALS, with a 1.5-fold higher disease rate for men. There are no race differences with either form of ALS. There is a higher prevalence of ALS in individuals older than 60 years, and the annual worldwide ALS mortality rate is 1 per 100,000.

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