Fitting J W, Paillex R, Hirt L, Aebischer P, Schluep M
Division de Pneumologie, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
Ann Neurol. 1999 Dec;46(6):887-93.
Impairment of pulmonary function is a major prognostic indicator in amyotrophic lateral sclerosis (ALS). Forced vital capacity (FVC) and maximal voluntary ventilation (MVV) decline linearly and are commonly used to assess disease progression. The aim of this study was to evaluate the usefulness of testing respiratory muscle strength in ALS with a novel test, sniff nasal pressure (Pn(sn)), in parallel with more classic tests such as maximal inspiratory pressure (PI(max)) and maximal expiratory pressure (PE(max)). Sixteen patients with ALS were examined monthly over a period of 18 +/- 10 months. At the time of inclusion in the study, values were normal for FVC (107% of predicted value) and MVV (87% of predicted value) but abnormally low for Pn(sn) (67% of predicted value), PI(max) (69% of predicted value), and PE(max) (54% of predicted value). Late in the course of ALS, all patients could perform Pn(sn) whereas 6 could not perform PI(max) and 7 could not perform PE(max). The rate of deterioration was most often linear and similar for FVC (-4.1% of predicted value per month), MVV (-4.3% of predicted value per month), and Pn(sn) (-4.2% of predicted value per month). We conclude that Pn(sn) was the single respiratory test combining linear decline, sensitivity in mild disease, and feasibility in advanced disease. Being easy to perform and inexpensive, Pn(sn) appears well suited to assess the decline of respiratory muscle strength in ALS.
肺功能损害是肌萎缩侧索硬化症（ALS）的主要预后指标。用力肺活量（FVC）和最大自主通气量（MVV）呈线性下降，常用于评估疾病进展。本研究的目的是通过一种新型测试——嗅鼻压力（Pn(sn)），与最大吸气压力（PI(max)）和最大呼气压力（PE(max)）等更经典的测试并行，评估在ALS中测试呼吸肌力量的有用性。16例ALS患者在18±10个月的时间里每月接受检查。在纳入研究时，FVC（预测值的107%）和MVV（预测值的87%）值正常，但Pn(sn)（预测值的67%）、PI(max)（预测值的69%）和PE(max)（预测值的54%）异常低。在ALS病程后期，所有患者都能进行Pn(sn)测试，而6例不能进行PI(max)测试，7例不能进行PE(max)测试。FVC（每月下降预测值的4.1%）、MVV（每月下降预测值的4.3%）和Pn(sn)（每月下降预测值的4.2%）的恶化率大多呈线性且相似。我们得出结论，Pn(sn)是唯一一项兼具线性下降、对轻症疾病敏感以及对重症疾病可行的呼吸测试。Pn(sn)操作简便且成本低廉，似乎非常适合评估ALS中呼吸肌力量的下降。
