Ventricular arrhythmia in X-linked emery-dreifuss muscular dystrophy: A lesson from an autopsy case

Abstract

Emery-Dreifuss muscular dystrophy (EDMD) is a distinctive form of muscular dystrophy which is often associated with cardiac abnormalities. Conduction disturbances are frequently observed, and may necessitate pacemaker implantation to prevent sudden death. In this case report, we present an autopsy of a 31-year-old man with X-linked EDMD who developed only minimal skeletal muscle symptoms, and who died from ventricular arrhythmia despite undergoing a previous pacemaker implantation. Ventricular arrhythmias in Xlinked EDMD patients are also discussed. © 2011 The Japanese Society of Internal Medicine.