Prospective incidence of ALS in a Canadian population (P4.135)

OBJECTIVE: To determine incidence of amyotrophic lateral sclerosis (ALS) in a geographically-contained, census-defined Canadian population. BACKGROUND: Incidence of ALS in Nova Scotia has been prospectively studied for approximately 30 years. Age-adjusted incidence per 100,000 was 2.13 in 2003, 2.3 in 1995, and 2.22 in 1984 using the direct method of age-standardization to the 2001 Canadian standardized population (1). DESIGN/METHODS: All neurologists and physiatrists in the province of Nova Scotia and neurologists in Moncton, New Brunswick (a city near the provincial border) were requested to report incident cases of ALS among Nova Scotia residents between May 1 2013 and April 30 2014. ALS was defined using the 1994 El Escorial criteria (2) in keeping with prior surveys conducted in Nova Scotia. Subtypes included classical ALS, progressive bulbar palsy, primary lateral sclerosis, and progressive muscular atrophy. RESULTS: Over the one year study period, there were 24 cases of ALS diagnosed among Nova Scotia residents. Mean age at diagnosis was 67 years with a slight male predominance (n=13:11 male:female). Classical ALS (n=19) was more common than progressive muscular atrophy (n=3), progressive bulbar palsy (n=1), or primary lateral sclerosis (n=1). Age-adjusted incidence per 100,000 of ALS was 1.94 (95[percnt] CI 1.1 to 2.7) during the study period using the direct method of age-standardization to the 2001 Canadian standardized population. CONCLUSIONS: Incidence of ALS in a geographically-contained, census-defined Canadian population has remained stable over approximately 30 years. REFERENCES: (1) Bonaparte JP, Grant IA, Benstead TJ, Murray TJ, Smith M. ALS incidence in Nova Scotia over a 20-year-period: A prospective study. Can J Neurol Sci. 2007;34:69-73. (2) Brooks BR. El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. J Neurol Sci. 1994;124(Suppl):96-107. Disclosure: Dr. Parks has nothing to disclose. Dr. Benstead has nothing to disclose. Dr. Grant has nothing to disclose.