Several epidemiologic studies of amyotrophic lateral sclerosis have shown a uniform repartition over the world. We report the results of a retrospective study and the preliminary results of a prospective study carried out in the Limousin area. The mean incidence per/year is 0.97/100,000 in the retrospective study and 1.3/100,000 in the prospective study. Such data are similar to those previously reported. The two major findings are the high frequency of bulbar palsy and the high mean age of the patients. This can be associated with the high mean age of the population in the Limousin area as compared to other regions.