报告强直性肌营养不良24例,其中21例分布在5个家系,3例散发;起病年龄2例在15岁以下,其余22例在20～28岁之间。临床表现除肌萎缩、肌强直外,尚有多系统病变的症状。肌电图检查有强直性电位。4例肌肉活检示不同程度的变性、萎缩,但神经末梢和运动终板完好,似不支持神经元发病机理之说。 Twenty-four cases of myotonic dystrophy were reported, of which 21 cases were distributed in 5 pedigrees and 3 cases were sporadic; the age of onset was 2 years under 15 years and the remaining 22 cases were between 20 and 28 years old. In addition to clinical manifestations of muscle atrophy, muscle rigidity, there are still many symptoms of systemic disease. Electromyography has tonic potentials. 4 cases of muscle biopsy showed varying degrees of degeneration, atrophy, but the nerve endings and motor end plates intact, does not seem to support the pathogenesis of neurons that.