Neurorehabilitation, Neurology Unit and Neurophysiology Unit, San Raffaele Scientific Institute, Milan, Italy.
Experimental Neuropathology Unit, Division of Neuroscience, Institute of Experimental Neurology (INSPE), San Raffaele Scientific Institute, Milan, Italy.
Eur J Neurol. 2024 Aug;31(8):e16316. doi: 10.1111/ene.16316. Epub 2024 May 8.
The use of multiple tests, including spirometry, arterial blood gas (ABG) analysis and overnight oximetry (OvOx), is highly recommended to monitor the respiratory function of patients with motor neuron disease (MND). In this study, we propose a composite score to simplify the respiratory management of MND patients and better stratify their prognosis.
We screened the clinical charts of 471 non-ventilated MND patients referred to the Neuro-rehabilitation Unit of the San Raffaele Scientific Institute of Milan (January 2001-December 2019), collecting spirometric, ABG and OvOx parameters. To evaluate the prognostic role of each measurement, univariate Cox regression for death/tracheostomy was performed, and the variables associated with survival were selected to design a scoring system. Univariate and multivariate Cox regression analyses were then carried out to evaluate the prognostic role of the score. Finally, results were replicated in an independent cohort from the Turin ALS Center.
The study population included 450 patients. Six measurements were found to be significantly associated with survival and were selected to design a scoring system (maximum score = 8 points). Kaplan-Meier analysis showed significant stratification of survival and time to non-invasive mechanical ventilation adaptation according to score values, and multivariate analysis confirmed the independent effect of the respiratory score on survival of each cohort.
Forced vital capacity, ABG and OvOx parameters provide complementary information for the respiratory management and prognosis of MND patients and the combination of these parameters into a single score might help neurologists predict prognosis and guide decisions on the timing of the implementation of different diagnostic or therapeutic approaches.
为了监测运动神经元病（MND）患者的呼吸功能，强烈推荐使用多项测试，包括肺活量测定、动脉血气分析（ABG）和夜间血氧饱和度（OvOx）。在这项研究中，我们提出了一种综合评分，以简化 MND 患者的呼吸管理，并更好地分层其预后。
我们筛选了米兰圣拉斐尔科学研究所神经康复科（2001 年 1 月至 2019 年 12 月）收治的 471 例未行机械通气的 MND 患者的临床病历，收集了肺活量测定、ABG 和 OvOx 参数。为了评估每项测量的预后作用，我们对死亡/气管切开术进行了单变量 Cox 回归，选择与生存相关的变量来设计评分系统。然后进行单变量和多变量 Cox 回归分析，以评估评分的预后作用。最后，在都灵 ALS 中心的独立队列中复制了结果。
研究人群包括 450 例患者。发现有 6 项测量与生存显著相关，被选来设计评分系统（最高得分为 8 分）。Kaplan-Meier 分析显示，根据评分值，生存和无创机械通气适应时间的分层有显著差异，多变量分析证实了呼吸评分对每个队列生存的独立影响。
用力肺活量、ABG 和 OvOx 参数为 MND 患者的呼吸管理和预后提供了互补信息，将这些参数组合成一个单一的评分可能有助于神经科医生预测预后，并指导不同诊断或治疗方法实施时机的决策。
