Survival in myotonic dystrophy type 1 predicted by the new DM1 survival risk score


A reliable prediction of survival in myotonic dystrophy type 1 (DM1) is critically important to plan a personalised health supervision. We retrospectively analysed the survival of 1,066 patients with genetically proven DM1 included in the DM1-Heart-Registry between January 2000 and November 2014 in Cochin and Pitié-Salpêtrière hospitals, assessed its predictors with multivariable Cox modelling, developed and internally validated a score to predict survival. We validated this score in an separate sample of 230 patients referred to the University Hospitals of Tours and Nantes.


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