萎缩性肌强直又称强直性肌营养不良或Steinert氏病,是一种少见的常染色体显性遗传的肌病(1)。本文报告一个家族的发病情况和五名经临床验证的病例及其肌电图变化;并简略讨论本病的发病机理。家系调查分析我们通过先证者(例1)倪某某及其部分家属,采用住院、门诊、信访或间接了解等方式,调查该家族四代中的发病数及其遗传方式。在四代23人中共发现11名患者,计男性9例,女性2例。四代中每代都有人患病,符合 Atrophic myotonia, also known as myotonic dystrophy or Steinert’s disease, is a rare, autosomal dominant myopathy (1). This article reports the incidence of a family and five clinically validated cases and their EMG changes; and briefly discusses the pathogenesis of this disease. Pedigree investigation and analysis We use probands (example 1) Ni Moumou and some of their family members, using inpatient, outpatient, petition or indirect understanding, etc., to investigate the incidence of the fourth generation of the family and its hereditary patterns. Eleven patients were found in 23 generations in four generations, accounting for 9 males and 2 females. Four generations in each generation are sick, in line