Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder of motor neurons in the cerebral cortex, brainstem and spinal cord. Although ALS is uncommon, accurate incidence information is needed to determine the impact of the disease on the health care system. Furthermore, regional differences in ALS incidence and changes in incidence over time may provide clues regarding the etiology of the disease.Numerous epidemiological studies of ALS have been conducted, most of which used retrospective methods. [1][2][3][4][5][6][7][8] Many of these studies analyzed large geographic areas with unstable populations, creating methodological challenges with respect to ABSTRACT: Objective: Previous studies have suggested that the incidence of amyotrophic lateral sclerosis (ALS) in Nova Scotia is relatively high and increasing over time. This study was performed to determine the current incidence of ALS in Nova Scotia and to compare this to data collected in 1984 and 1995. Methods: All physiatrists and neurologists were surveyed on a monthly basis over one year to record all new cases of ALS diagnosed in Nova Scotia. Data was compared to that collected using similar methods in 1984 and 1995. To validate our methods, we also performed a retrospective study using a provincial health care database. Results: There were 21 new ALS cases in Nova Scotia during the 2003 study period, yielding a crude incidence of 2.24/100,000. The age-adjusted incident rate for 2003 was 2.13 (95% CI = 0.11-4.15). The age-adjusted rate for 1995 was 2.3 (95% CI = 0.08-4.53) while the age-adjusted rate for 1984 was 2.22 (95% CI = 0.13-4.32). Analysis of provincial health records identified 24 cases of ALS and an age-adjusted incidence of 2.44/100,000. Conclusions: The ageadjusted incidence of ALS in Nova Scotia has remained stable over the period 1984-2003. The incidence is similar to that reported in several other parts of the world.