肌萎缩侧索硬化起病部位与通气功能障碍的关系

Association between site of onset and ventilation dysfunction in patients with amyotrophic lateral sclerosis

目的 采用回顾性研究的方法探讨中国肌萎缩侧索硬化（ALS）的起病部位与通气功能障碍的关系.方法 采集2009年1月至2013年9月北京大学第三医院神经内科诊治的344例散发性ALS（SALS）的临床病例,比较不同起病部位的患者之间用力肺活量（FVC）的差异.结果 在344例SALS患者中,球部起病组55例,FVC均值为84％±14％,FVC＜ 80％有24例（43.64％）.上肢起病组200例,FVC均值为83％±17％,FVC＜ 80％有81例（40.50％）.下肢起病组89例,FVC均值为88％±16％,FVC＜ 80％有25例（28.09％）.上肢起病组的FVC均值显著低于下肢起病组（P＜0.05）,且发生FVC＜ 80％的比例显著高于下肢起病组（P＜0.05）.球部起病组的FVC均值略低于下肢起病组,发生FVC＜ 80％的比例高于下肢起病组,但差异无统计学意义.结论 与上肢起病的SALS相比,下肢起病的患者更少且更晚出现通气功能障碍,也更少及更晚需要应用无创正压通气（NIPPV）治疗.

Objective To study the association between site of onset and ventilation dysfunction in Chinese patients with amyotrophic lateral sclerosis.Methods The clinical data of 344 patients with sporadic amyotrophic lateral sclerosis （SALS） in Department of Neurology of Peking University Third Hospital from January 2009 to September 2013 were retrospectively reviewed.The differences of Forced Vital Capacity （FVC） between groups with distinct site of onset were analyzed.Results There were 55, 200 and 89 patients in the group with bulbar, upper limb and lower limb onset, respectively.The values of FVC were 84%＆#177;14%, 83% ＆#177; 17% and 88% ＆#177; 16%, and the cases of FVC 〈 80% were 24 （43.64%）, 81 （40.50%） and 25 （28.09%） in each group.The mean value of FVC was significantly lower and the frequency of FVC 〈 80% statistically higher in the group with upper limb onset in comparison with the group with lower limb onset.The mean value of FVC was lower and the frequency of FVC 〈 80% higher in the group with bulbar onset than in the group with lower limb onset.However, the differences were not significant between the two groups.Conclusions Compared with SALS with upper limb onset, ventilation function disorder may occur less and later in patients with lower limb onset, who may also require noninvasive positive pressure ventilation （NIPPV） less and later.