221例散发型肌萎缩侧索硬化症的临床特征
Clinical characteristics of 221 cases of sporadic amyotrophic lateral sclerosis

摘要：目的 总结西安交通大学第一附属医院确诊的221例肌萎缩侧索硬化症(ALS)患者的临床特点，提高对ALS的临床认识。方法 2009年1月至2015年12月于西安交通大学第一附属医院神经内科确诊的ALS患者，每3～6个月随访1次，至少随访2年，记录用药及病情变化情况。结果 纳入221例ALS患者，男女比为1.45∶1，平均起病年龄(54.12±10.39)岁，51～60岁年龄段患者最多，有81例(36.65%)；起病年龄与病程呈负相关(r=－0.185，P=0.013)；34例(15.38%)延髓起病，187例(84.62%)脊髓起病，不同起病部位之间性别(P=0.007)、病程(P=0.048)分布差异具有统计学意义；平均诊断延迟时间为(15.22±13.65)月；45例(20.36%)ALS患者服用利鲁唑，平均服用时间为(13.79±12.69)月，其病程(月)长于非利鲁唑治疗组(48.38±3.25 vs. 39.62±2.42，P=0.034)。结论 221例ALS患者以脊髓起病多见，特别是男性患者；相比中国其他地区，诊断延迟时间更长、利鲁唑使用率更低，这与西北地区经济相对落后、医疗条件相对较差有关。
ABSTRACT: Objective? ?To analyze the clinical characteristics of sporadic amyotrophic lateral sclerosis (ALS) so as to improve our understanding of ALS. Methods?? We included the patients with sporadic ALS diagnosed by the Department of Neurology of the First Affiliated Hospital of Xi’an Jiaotong University during January 2009 and December 2015, followed them up every 3-6 months for at least 2 years, and recorded their medication and disease progression. Results?? There were 221 ALS patients recruited in this study. The male-to-female ratio was 1.45∶1. The mean age of ALS onset was (54.12±10.39) years old and the peak age of onset was in the 51 to 60 year-old group with 81 patients (36.65%). We found a negative correlation between the age of onset and course of ALS (r=－0.185, P= 0.013). There were 34 patients (15.38%) with inital bulbar symptoms and 187 patients (84.62%) with inital spinal symptoms. The region of onset showed significant difference with gender (P=0.007) and course of ALS (P=0.048). The mean time delay from symptom onset was (15.22±13.65) months. There were 45 ALS patients (20.36%) treated by riluzole and the mean time of treatment was 13.79±12.69 months. The course of ALS patients treated with riluzole [(48.38±3.25) months] was longer than that of ALS patients without riluzole treatment [(39.62±2.42) months, P=0.034]. Conclusion? ?ALS patients, especially male patients, showed inital spinal symptoms more often. The time delay from symptom onset was longer and usage of riluzole was lower than that in other regions of China, which might be caused by the economic backwardness and poor medical condition of Northwest China