作者等于1955～1980年间共收集了符合诊断标准的进行性脊肌萎缩症(PMA)155例,进行长期随访及其生存分析,目的在于观察若干临床征象对其病程的影响,以及在PMA明确诊断后,如何估价其预后。本组男121例,女34例,男女之比为3.56∶1,平均年龄为48.4岁。最先受累的部位:手部73例(47.1%),肩胛带35例(22.6%),下肢47例(30.3%),本组无1例出现骨盆带肌肉及呼吸肌受累。肌束震颤89例(57.4%)。根据肌萎缩、肌束震颤的范围及电生理检查结果,又分为“局限型”和“弥漫型”两类。在长期随访中,除7例失去联系外,计存活67例,死亡81例。在PMA初次诊断后3年内,存活率迅速下降,此后存活率下降较缓慢。3年及5年内存活率的统计分别为61.3%和56.4%。本组存活10年以上者16例,15年以上者10例,有1例存活长 The authors enrolled 155 patients with progressive spinal muscular atrophy (PMA) who met the diagnostic criteria from 1955 to 1980 for long-term follow-up and survival analysis with the aim of observing the impact of several clinical signs on their course of disease and on a clear diagnosis of PMA After how to assess the prognosis. The group of 121 males and 34 females, male to female ratio was 3.56: 1, with an average age of 48.4 years. The first site involved: 73 cases (47.1%) in the hand, 35 cases (22.6%) in the shoulder girdle and 47 cases (30.3%) in the lower limb. None of the patients in this group had pelvic muscle and respiratory muscle involvement. 89 cases of fasciculation (57.4%). According to muscle atrophy, the scope of muscle bundle tremor and electrophysiological examination results, is divided into “localized” and “diffuse” two categories. In the long-term follow-up, in addition to the seven cases of loss of contact, the survival of 67 cases, 81 patients died. In 3 years after the initial diagnosis of PMA, the survival rate decreased rapidly, after which the survival rate decreased more slowly. The three-year and five-year survival rates were 61.3% and 56.4%, respectively. In this group, 16 cases survived more than 10 years, 10 cases more than 15 years, and 1 case survived