Risk of arrhythmia in type I myotonic dystrophy: The role of clinical and genetic variables

P. Cudia, P. Bernasconi, R. Chiodelli, F. Mangiola, F. Bellocci, A. Dello Russo, C. Angelini, V. Romeo, P. Melacini, L. Politano, A. Palladino, G. Nigro, G. Siciliano, M. Falorni, M. G. Bongiorni, C. Falcone, R. Mantegazza, L. Morandi

Research output: Contribution to journalArticlepeer-review


Objective: To examine the association between the presence of arrhythmia in type 1 myotonic dystrophy (DM1) and clinical-genetic variables, evaluating their role as predictors of the risk of arrhythmia. Methods: 245 patients with genetically proven DM1 underwent clinical and non-invasive cardiological evaluation. Severity of muscular involvement was assessed according to the 5 point Muscular Disability Rating Score (MDRS). Data were analysed by univariate and multi-variate models. Results: 245 patients were examined and cardiac arrhythmias were found in 63 subjects, 40 of whom required a device implant. Statistical analyses revealed that men had more than double the risk of developing arrhythmias compared with women (p = 0.018). Addition of each year of age caused an increased risk of arrhythmia equal to 3% (p = 0.030). Subjects with MDRS 5 had a risk of arrhythmia 12 times higher than patients with MDRS 1-2 (p

Original languageEnglish
Pages (from-to)790-793
Number of pages4
JournalJournal of Neurology, Neurosurgery and Psychiatry
Issue number7
Publication statusPublished - Jul 2009

ASJC Scopus subject areas

  • Clinical Neurology
  • Psychiatry and Mental health
  • Surgery


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