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Background and purpose The main objective of establishing the French register of amyotrophic lateral sclerosis ( ALS) in the Limousin region ( FRALim), was to assess the incidence of ALS, in this ageing region of Europe, over a 12-year period (2000-2011). Methods Patients were included if they lived in Limousin at the time of diagnosis of ALS according to El Escorial revised criteria and were identified by at least one of the following sources: (i) the French national body coordinating ALS referral centres; (ii) public and private hospitals in the region; (iii) health insurance data related to long-term diseases. Results The FRALim register identified 279 incident cases (2000-2011). The crude and European population standardized incidences of ALS were as high as 3.19/100 000 person-years of follow-up (95% CI 2.81-3.56) and 2.58/100 000 person-years of follow-up (95% CI 2.27-2.89) respectively. Median age at onset was 70.8 years (interquartile range 63.1-77.1). The standardized sex incidence ratio (male/female) was 1.3 overall, but 1.1 under the age of 65 years, 1.7 between 65 and 75 years and 1.9 above 75 years. The exhaustiveness of the register has been estimated at 98.4% (95% CI 95.6-99.4) by capture-recapture analysis. Conclusion It was possible for the first time in France to monitor accurately the incidence of ALS over a long time period. It appears to be in the upper range of data reported in western countries. Patterns displayed here might anticipate the epidemiology of ALS in ageing western countries. [ABSTRACT FROM AUTHOR]