title: Sudden cardiac death in myotonic dystrophy type 2.
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.
authors.affiliation: Friedrich Baur Institute, Department of Neurology, Ludwig Maximilians University Munich, Ziemssenstr. 1a, 80336 Munich, Germany. bschoser@med.uni-muenchen.de
authors.forename: B G H
authors.initials: BG
authors.lastname: Schoser
authors.forename: K
authors.initials: K
authors.lastname: Ricker
authors.forename: C
authors.initials: C
authors.lastname: Schneider-Gold
authors.forename: C
authors.initials: C
authors.lastname: Hengstenberg
authors.forename: J
authors.initials: J
authors.lastname: Dürre
authors.forename: B
authors.initials: B
authors.lastname: Bültmann
authors.forename: W
authors.initials: W
authors.lastname: Kress
authors.forename: J W
authors.initials: JW
authors.lastname: Day
authors.forename: L P W
authors.initials: LP
authors.lastname: Ranum
mesh_terms: D000328:Adult; D002037:Bundle-Branch Block / Q000209:etiology / Q000473:pathology; D002311:Cardiomyopathy, Dilated / Q000209:etiology* / Q000473:pathology; D002893:Chromosomes, Human, Pair 3 / Q000235:genetics*; D016757:Death, Sudden, Cardiac / Q000453:epidemiology*; D005260:Female; D005355:Fibrosis; D005500:Follow-Up Studies; D020022:Genetic Predisposition to Disease; D006329:Heart Conduction System / Q000473:pathology; D006333:Heart Failure / Q000209:etiology* / Q000473:pathology; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D020766:Intracranial Embolism / Q000209:etiology / Q000473:pathology; D008297:Male; D018895:Microsatellite Repeats; D009206:Myocardium / Q000737:chemistry / Q000473:pathology*; D009223:Myotonic Dystrophy / Q000145:classification / Q000150:complications* / Q000235:genetics; D012313:RNA / Q000032:analysis*; D012306:Risk
chemical_list: D012313:RNA