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Sudden cardiac death in myotonic dystrophy type 2
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Sudden cardiac death in myotonic dystrophy type 2 NEUROLOGY Schoser, B. G., Ricker, K., Schneider-Gold, C., Hengstenberg, C., Durre, J., Bultmann, B., Kress, W., Day, J. W., Ranum, L. P. 2004; 63 (12): 2402-2404Abstract
Medical records and follow-up data were reviewed in 297 genetically proven myotonic dystrophy type 2 (DM2) patients. Patients were selected by the criteria of cardiac sudden death before age 45. Sudden death occurred in four patients, three of whom were cardiological asymptomatic, and one with a history of heart failure. Cardiac histopathology showed dilated cardiomyopathy in all, and conduction system fibrosis in two patients. Pathogenetic CCUG ribonuclear inclusions were demonstrable in cardiomyocytes.
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