Sudden cardiac death in myotonic dystrophy type 2

Autor: Schoser, B G.H., Ricker, K, Schneider-Gold, C, Hengstenberg, C, Dürre, J, Bültmann, B, Kress, W, Day, J W., Ranum, L P.W.
Zdroj: Neurology (Ovid); December 2004, Vol. 63 Issue: 12 p2402-2404, 3p
Abstrakt: 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.
Databáze: Supplemental Index