【摘要】 [目的]研究连枷臂(FAS)和连枷腿(FLS)综合征的病程发展特点和临床特征,提高对它们的识别能力。[方法]将325例临床确诊和临床可能诊断级别的经典肌萎缩侧索硬化(ALS)患者的临床资料进行整理分析,并与同期诊断的26例FAS患者与7例FLS患者的人口学资料、临床特征和生存情况进行比较。[结果]ALS患者中肢体起病者的平均症状扩展时间间隔为(12.5±3.2)个月,平均生存时间为(47.2±4.4)个月,5年生存率为28.3%。而FAS患者的平均症状扩展时间为(49.6±6.8)个月,平均生存时间和5年生存率分别为(67.3±6.2)个月和69.4%,FAL患者的以上数据分别为(48.1±9.9)个月、(66.2±7.9)个月和85.7%,FAS和FLS与肢体起病的ALS在症状扩展时间、平均生存时间和5年生存率均存在统计学差异。经典ALS男女比例为1.5︰1,FAS男女比例为4.2︰1,而FLS的男女比例是6︰1,FAS和FLS的男女比例明显高于经典ALS,达到统计学意义上的差异。[结论]FAS和FLS是病程发展相对缓慢和生存时间更长的ALS变异型。这对更好的判断ALS患者的预后及制订合理治疗方案具有重要意义。更多还原

【Abstract】 [Objective]To investigate the clinical features and properties of medical history of ALS variants-flail arm syndrome and flail leg syndrome.[Methods]We analyzed clinical data of 358 cases with definite or probable ALS.There were 325 patients with typical ALS and 26 patients with FAS and 7 cases with FAL in our cohort.Then we compared the demographic characteristics and five-year survival rate as well as average survival time in groups of classical ALS,FAS and FAL.[Results]In our cohort,average survival was 47.2±4.4 months for limb onset ALS,whereas 67.3±6.2 months for FAS and 66.2±7.9 months for FLS.Five-year survival rate was 28.3% for limb onset ALS,69.4% for FAS,and 85.7% for FLS.There were statistic differences between typical limb onset ALS and group of FAS and FLS in above parameters.The ratio of men to women was 4.2:1 in the FAS group compared to 1.5:1 in limb onset ALS cases.[Conclusion]FAS and FAL have significantly better survival time than typical amyotrophic lateral sclerosis(ALS) .Recognizing FAS and FLS has great importance to make treatment plan for patients with ALS.更多还原