Table 3.

Distribution of observed and expected deaths and 10-year cumulative risk of death by myotonic dystrophy (DM) phenotypes and muscular weakness groups

Baseline no. patientsDeathsRatio10-year cumulative risk (%)
Observed (O)Expected (E)*O/E95% CI
* Matched general population of the same age; based on 1987–1990 Quebec life tables (Bureau de la Statistique du Québec).
DM phenotype
Mild2262.42.50.9–4.927.3
Adult99292.610.77.2–14.929.3
Early adult134182.08.95.2–13.413.4
Childhood3040.314.53.8–32.213.3
Congenital1800.10.0
Muscular weakness
No weakness7552.61.90.6–3.96.7
Distal weakness96151.69.25.1–14.515.6
Proximal weakness92333.010.97.5–14.935.9