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Sudden cardiac death in myotonic dystrophy type 2

B. G.H. Schoser, MD, K. Ricker, MD, C. Schneider-Gold, MD, C. Hengstenberg, MD, J. Dürre, MD, B. Bültmann, MD, W. Kress, PhD, J. W. Day, MD, PhD and L. P.W. Ranum, PhD

From the Friedrich Baur Institute (Dr. Schoser), Department of Neurology, Ludwig-Maximilians-University of Munich; the Departments of Neurology (Drs. Ricker and Schneider-Gold) and Human Genetics (Dr. Kress), University of Würzburg; the Department of Cardiology (Dr. Hengstenberg), University of Regensburg; Department of Pathology (Dr. Dürre), Ludwigsburg; Institute of Pathology (Dr. Bültmann), University of Tübingen, Germany; the Department of Neurology (Dr. Day), Institute of Human Genetics (Drs. Day and Ranum); and the Department of Genetics, Cell Biology, and Development (Dr. Ranum), University of Minnesota, Minneapolis.

Address correspondence and reprint requests to Dr. Benedikt G.H. Schoser, Friedrich Baur Institute, Department of Neurology, Ludwig Maximilians University Munich, Ziemssenstr. 1a, 80336 Munich, Germany; e-mail: bschoser{at}med.uni-muenchen.de

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.

Received June 3, 2004. Accepted in final form August 9, 2004.

Deceased.

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