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强直性肌营养不良症是成人中最常见的肌营养不良,发生率为5/10万。本病除有神经肌肉表现外,尚有多系统受累,包括白内障、精神发育迟缓及心脏损害。1967年的一项研究证实,236例该病患者中,心电图异常者202例,但仅16%有心脏症状,其中一半症状是由心律失常引起的。明显的原发性心肌损害罕见,偶有报道亚急性扩张型心肌病及局灶性心肌炎,但多无临床症状。Hartwig 等报道10例患者中于运动时出现室壁运动异常。本病至少30%患者出现二尖瓣脱垂。心脏的组织学特征包括纤维化及脂
Myotonic dystrophy is the most common form of muscular dystrophy in adults at a rate of 5 / 100,000. Apart from neuromuscular manifestations of the disease, there are many systemic involvement, including cataracts, mental retardation and heart damage. A 1967 study confirmed that 236 of 236 patients had an abnormal electrocardiogram in 202 patients, but only 16% had cardiac symptoms, and half of them were caused by cardiac arrhythmias. Obvious primary myocardial damage rare, occasional sub-acute dilated cardiomyopathy and focal myocarditis, but no clinical symptoms. Hartwig et al reported that 10 cases of patients with exercise-induced wall motion abnormalities. Mitral valve prolapse occurs in at least 30% of patients with this disease. Histological features of the heart include fibrosis and lipids