Demographic characteristics and prognosis in a Flemish Amyotrophic LateralSclerosis population

We describe the genetic and demographic characteristics of patients with am yotrophic lateral sclerosis (ALS) in Flanders, Belgium. Prognostic factors related to survival are examined. ALS was familial in 8.6% of all MND patients. In 6 of 8 apparently unrelate d families, an SOD1 mutation was found. In sporadic ALS, mean age at onset was 57.1 years. There was a male preponderance (1.2:1) and the disease had a bulbar onset in 19%. Median survival was 32 months (95% CI 26-46). The pr esence of an APOE-epsilon 4 allele was nor associated with a bulbar onset o f ALS; an earlier age at onset or a shorter median survival Variables exami ned in a multivariate analysis included age, sex, site of onset, delay from onset to diagnosis, and % forced vital capacity Shorter survival was indep endently associated with higher age, bulbar onset, a short diagnostic delay , and a lower percent-predicted vital capacity at study entry. Simple clinical baseline characteristics can assist the clinician in estima ting prognosis in ALS. The demographic characteristics of the Flemish ALS p opulation do not seem to differ from those described in other parts of the world.