Our objective was to describe amyotrophic lateral sclerosis (ALS) mortality rates in the Chilean population over a 17-year period. Chilean death records (1994-2010) were reviewed for the ICD-10 diagnosis G.12.2 (including motor neuron disease and similar conditions), and weighted with population data. Crude and standardized mortality rates by ALS were calculated at the nationwide level and by geographic zone. A risk analysis was performed in successive cohorts from 1910-1919 to 1960-1969, comparing mortality slopes. One thousand six hundred and seventy-one deaths were recorded during 1994-2010, with an average of 1.13 per 100,000, a 1.2:1 male/female ratio, and a statistically significant increase in mortality rate. According to geographical distribution, the Austral area, with a larger population of European origin, showed higher mortality rates compared to the national average. The cohort analysis showed an increasing risk of dying from ALS for all cohorts, and highest above 64 years of age, becoming a competitive cause of death in older ages. In conclusion, as expected, the mortality rate in Chile by ALS is higher than that reported previously in our country, and similar to other Latin American countries. ALS mortality rate has increased over time probably due to the aging of the population and decline in rates for competing causes of death.
Keywords: Amyotrophic lateral sclerosis; mortality rates; motor neuron disease.