Gay P C, Westbrook P R, Daube J R, Litchy W J, Windebank A J, Iverson R
Division of Thoracic Diseases, Mayo Clinic, Rochester, MN 55905.
Mayo Clin Proc. 1991 Jul;66(7):686-94. doi: 10.1016/s0025-6196(12)62080-1.
Breathing abnormalities and nocturnal hypoventilation occur in patients with amyotrophic lateral sclerosis (ALS). A prospective study was undertaken to determine the relationship of pulmonary function test abnormalities with quality of sleep and survival in 21 patients with ALS. Results of spirometry including determination of maximal respiratory pressures and arterial blood gases were compared with several formal polysomnographic variables and then also with 18-month survival. The patients had mild to moderate pulmonary function deficits, but the quality of sleep was best related to age (mean age, 58.5 years). The results of pulmonary function tests and arterial blood gas measurements did not correlate well with the presence of nocturnal breathing events or survival time, but the maximal inspiratory pressure was 86% sensitive for predicting the presence of a nocturnal oxygen saturation nadir of 80% or less and 100% sensitive for predicting 18-month survival. Although obstructive breathing events occurred, the primary explanation for the decline in nocturnal oxygen saturation was hypoventilation. We conclude that routine pulmonary function tests may be useful for screening for reductions in nocturnal oxygen saturation and also may have prognostic value. Further studies may determine whether treatment of nocturnal hypoventilation will have an effect on survival in patients with ALS who have breathing impairment.
肌萎缩侧索硬化症（ALS）患者会出现呼吸异常和夜间通气不足。一项前瞻性研究旨在确定21例ALS患者肺功能测试异常与睡眠质量及生存率之间的关系。将肺活量测定结果（包括最大呼吸压力测定和动脉血气分析）与多个正式的多导睡眠图变量进行比较，然后再与18个月生存率进行比较。这些患者存在轻度至中度肺功能缺陷，但睡眠质量与年龄（平均年龄58.5岁）的相关性最强。肺功能测试结果和动脉血气测量结果与夜间呼吸事件的存在或生存时间并无良好的相关性，但最大吸气压力对预测夜间氧饱和度最低点为80%或更低的存在的敏感度为86%，对预测18个月生存率的敏感度为100%。尽管存在阻塞性呼吸事件，但夜间氧饱和度下降的主要原因是通气不足。我们得出结论，常规肺功能测试可能有助于筛查夜间氧饱和度降低情况，也可能具有预后价值。进一步的研究或许能确定对夜间通气不足的治疗是否会对存在呼吸障碍的ALS患者的生存率产生影响。
