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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): 2402-2404

Abstract

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.

View details for Web of Science ID 000226010000039

View details for PubMedID 15623712