Epidemiology of myotonic dystrophy type 1 (Steinert disease) in Belgrade (Serbia)

Abstract The aim of this study was to estimate the incidence and prevalence of myotonic dystrophy type 1 (DM1) in Belgrade during the period 1983–2002. The patients who had DM1 were ascertained through hospital records from all neurological departments in Belgrade during 1983–2002. The molecular genetic analysis was performed in all patents included in the study. We identified 101 DM1 patients (52 males and 49 females). The average annual incidence rate of DM1 in Belgrade for the period observed was 2.0/1,000,000 (95% confidence interval (CI), 0.3–8.3), 2.1/1,000,000 (95% CI, 0.3–8.3) for males and 2.0/1,000,000 (95% CI, 0.3–8.3) for females. The highest age-specific DM1 incidence was registered in the age group 20–49: 3.4/1,000,000 (95% CI, 0.5–7.6), 4.0/1,000,000 (95% CI, 1.1–10.2) in males and 2.5/1,000,000 (95% CI, 0.5–7.6) in females. In the population of Belgrade, a cumulative probability of acquiring DM1 was 1 per 8621 for men and 1 per 9259 for women (1 per 8940 of the population for both sexes). The prevalence of DM1 in Belgrade on 31 December 2002 was 5.3/100,000 (95% CI, 4.2–6.6).