Capozzo Rosa, Quaranta Vitaliano N, Pellegrini Fabio, Fontana Andrea, Copetti Massimiliano, Carratù Pierluigi, Panza Francesco, Cassano Anna, Falcone Vito A, Tortelli Rosanna, Cortese Rosa, Simone Isabella L, Resta Onofrio, Logroscino Giancarlo
Neuroscience and Sense Organs, Department of Basic Medical Science, Neurodegenerative Diseases Unit, University of Bari "Aldo Moro", Bari, Italy.
J Neurol. 2015 Mar;262(3):593-603. doi: 10.1007/s00415-014-7613-3. Epub 2014 Dec 19.
Forced vital capacity (FVC) shows limitations in detecting respiratory failure in the early phase of amyotrophic lateral sclerosis (ALS). In fact, mild-to-moderate respiratory muscle weakness may be present even when FVC is normal, and ALS patients with bulbar involvement might not be able to perform correctly the spirometry test. Sniff nasal inspiratory pressure (SNIP) is correlated with transdiaphragmatic strength. We evaluated SNIP at baseline as a prognostic factor of tracheostomy or death in patients with ALS. In a multidisciplinary tertiary care center for motorneuron disease, we enrolled 100 patients with ALS diagnosed with El Escorial criteria in the period between January 2006 and December 2010. Main outcome measures were tracheostomy or death. RECursive Partitioning and AMalgamation (RECPAM) analysis was also used to identify subgroups at different risks for the tracheostomy or death. Twenty-nine patients with ALS reached the outcome (12 died and 17 had tracheostomy). Using a multivariate model SNIP correctly classified the risk of the composite event within 1 year of follow-up with a continuous Net Reclassification Improvement cNRI of 0.58 (p = 0.03). Sex, Amyotrophic Lateral Sclerosis Functional Rating Scale revisited, site of onset, and FVC did not improve the classification of prognostic classes. SNIP ≤18 cmH2O identified the RECPAM class with the highest risk (Class 1, hazard ratio = 9.85, 95 % confidence interval: 2.67-36.29, p < 0.001). SNIP measured at baseline identified patients with ALS with initial respiratory failure. Finally, using only ALS patients with spinal onset of the disease, our findings were mostly overlapping with those reported in the models including the whole sample. At baseline, SNIP appeared to be the best predictor of death or tracheostomy within 1 year of follow-up. The measurement of SNIP in the early phase of the disease may contribute to identify patients with high risk of mortality or intubation. SNIP may also provide an additional tool for baseline stratification of patients with ALS in clinical trials.
用力肺活量（FVC）在检测肌萎缩侧索硬化症（ALS）早期呼吸衰竭方面存在局限性。事实上，即使FVC正常，也可能存在轻度至中度呼吸肌无力，并且延髓受累的ALS患者可能无法正确进行肺活量测定。嗅吸鼻吸气压力（SNIP）与跨膈强度相关。我们将基线时的SNIP评估为ALS患者气管切开术或死亡的预后因素。在一家运动神经元疾病多学科三级护理中心，我们纳入了2006年1月至2010年12月期间根据埃尔埃斯科里亚尔标准诊断为ALS的100例患者。主要结局指标为气管切开术或死亡。还使用递归划分与合并（RECPAM）分析来识别气管切开术或死亡风险不同的亚组。29例ALS患者达到了该结局（12例死亡，17例接受了气管切开术）。使用多变量模型，SNIP在随访1年内正确分类了复合事件的风险，连续净重新分类改善cNRI为0.58（p = 0.03）。性别、修订的肌萎缩侧索硬化功能评定量表、发病部位和FVC并未改善预后类别的分类。SNIP≤18 cmH2O确定了RECPAM中风险最高的类别（1类，风险比= 9.85，95%置信区间：2.67 - 36.29，p < 0.001）。基线时测量的SNIP可识别出初始存在呼吸衰竭的ALS患者。最后，仅使用疾病首发于脊髓的ALS患者，我们的研究结果与包含整个样本的模型中报告的结果大多重叠。在基线时，SNIP似乎是随访1年内死亡或气管切开术的最佳预测指标。在疾病早期测量SNIP可能有助于识别具有高死亡或插管风险的患者。SNIP还可为临床试验中ALS患者的基线分层提供额外工具。
