TY - T1的比较不同的呼吸测试的性能参数检测早期呼吸功能不全患者的肌萎缩性侧索硬化症JF -神经学乔-神经病学SP - e743 LP - e750做- 10.1212 / WNL。首页0000000000200758六世- 99 - 7非盟-塞西莉亚·凯利盟卡Parra-Cantu盟博因顿布拉德利- Prabin Thapa盟盟-伯纳德j·斯莱姆盟埃里克·j·索伦森AU -珍妮弗·m·Martinez-Thompson盟Jaywant Mandrekar AU -内森p .员工Y1 - 2022/08/16 UR - //www.ez-admanager.com/content/99/7/e743.abstract N2 -背景和目标比较不同呼吸功能测试的性能参数在一个多学科的肌萎缩性脊髓侧索硬化症(ALS)诊所。首页方法统计、临床数据和呼吸测试参数抽象从病人的医疗记录参加了一个多学科ALS诊所从2008年到2016年。We compared the performance of the 3 primary respiratory test parameters used by Medicare for the initiation of noninvasive ventilation (NIV) (forced vital capacity [FVC] < 50% predicted, maximum inspiratory pressure [MIP] < 60 cm H2O, and abnormal overnight pulse oximetry [OvOx]) on how they related to several clinically relevant attributes.Results Four hundred seventy-six patients were identified who underwent at least 1 respiratory test. Abnormalities of OvOx, MIP, and FVC occurred at a median of 1.6, 1.5, and 3.8 years from disease onset, respectively (p < 0.00001). Patients with bulbar-onset ALS exhibited earlier abnormalities in MIP and FVC than in spinal-onset ALS (p < 0.005). The median survival after an abnormal OvOx, MIP, or FVC test was 1.4, 1.4, and 0.9 years, respectively (p < 0.0001). Using the ALS Functional Rating Score respiratory subscales, at the time of reported respiratory symptoms there were abnormalities in OvOx (60%), MIP (69%), and FVC (19%). Conversely, when respiratory parameter abnormalities preceded reported respiratory symptoms, this occurred with frequencies in OvOx (79%), MIP (42%), or FVC (24%). Four hundred forty-three patients (93.1%) developed at least 1 abnormal respiratory measure meeting Medicare criteria for NIV consideration, but fewer than 50% in our cohort demonstrated NIV use. Improved survival in subjects using NIV was statistically significant in patients with bulbar-onset ALS.Discussion Abnormalities in OvOx and MIP perform better than FVC at early detection of neuromuscular respiratory weakness in ALS. Initiation of NIV in patients with respiratory insufficiency may improve the overall survival in ALS. In the setting of the COVID-19 pandemic, FVC and MIP have not been routinely performed because of infectious aerosol generation. OvOx, which we now routinely mail to patients' homes, has been used exclusively during the COVID-19 pandemic and allows for continued remote monitoring of the respiratory status of patients with ALS.Classification of Evidence This cohort study provides Class III evidence that in people with ALS, OvOx and MIP are valuable respiratory parameters for the detection of early respiratory insufficiency.ALS=amyotrophic lateral sclerosis; ALSFRS-R=Revised Amyotrophic Lateral Sclerosis Functional Rating Scale; FVC=forced vital capacity; MIP=maximum inspiratory pressure; NIV=noninvasive ventilation; OvOx=overnight pulse oximetry; SaO2=oxygen saturation ER -