Department of Neurosciences and Mental Health, Hospital de Santa Maria-CHLN, Lisbon, Portugal.
Faculdade de Medicina, Instituto de Medicina Molecular, Translational Clinical Physiology, Universidade de Lisboa, Lisbon, Portugal.
J Neurol. 2020 Jul;267(7):2123-2129. doi: 10.1007/s00415-020-09818-z. Epub 2020 Apr 6.
To evaluate phrenic nerve motor amplitude (PhrenicAmp) as an independent predictor of functional decline in amyotrophic lateral sclerosis (ALS). We also assessed both PhrenicAmp and forced vital capacity (FVC) as predictors of functional loss in patients with bulbar dysfunction.
We included consecutive ALS patients with PhrenicAmp and FVC at baseline. Participants were evaluated with the revised ALS Functional Rating Scale (ALSFRS-R) at inclusion and at, at least, one subsequent follow-up visit. The outcome measure of functional decline was the percentage reduction in ALSFRS-R from baseline. Bulbar dysfunction was defined by the presence of any relevant symptom on the ALSFRS-R bulbar sub-score. Correlations and mixed-effects regressions were used to study the relationship between functional decline and both PhrenicAmp and FVC baseline evaluations.
A total of 249 ALS patients were included; 64.2% of these had bulbar dysfunction. At inclusion, significant correlations were found between PhrenicAmp and FVC (p < 0.001), as well as between each respiratory measure and ALSFRS-R (all p < 0.001). The functional decline at first (median 3 months) and second (median 6 months) follow-up visits was significantly correlated with baseline values of both respiratory evaluations (all p < 0.01) in the entire ALS population, but only with baseline PhrenicAmp (all p < 0.05) in bulbar dysfunction cases. Regression analysis revealed that PhrenicAmp (all p < 0.05), but not FVC, was a significant independent predictor of functional decline in ALS patients and in those with bulbar dysfunction.
Baseline PhrenicAmp is an independent predictor of functional decline in ALS, whether or not bulbar dysfunction is present.
评估膈神经运动幅度（PhrenicAmp）作为肌萎缩侧索硬化症（ALS）功能下降的独立预测因子。我们还评估了 PhrenicAmp 和用力肺活量（FVC）作为球部功能障碍患者功能丧失的预测因子。
我们纳入了基线时具有 PhrenicAmp 和 FVC 的连续 ALS 患者。参与者在纳入时和至少一次后续随访时使用修订后的 ALS 功能评定量表（ALSFRS-R）进行评估。功能下降的结局测量是从基线到 ALSFRS-R 的百分比降低。球部功能障碍定义为 ALSFRS-R 球部子量表上存在任何相关症状。相关性和混合效应回归用于研究功能下降与 PhrenicAmp 和 FVC 基线评估之间的关系。
共纳入 249 例 ALS 患者；其中 64.2%有球部功能障碍。在纳入时，发现 PhrenicAmp 和 FVC 之间存在显著相关性（p<0.001），以及每个呼吸测量与 ALSFRS-R 之间存在显著相关性（均 p<0.001）。整个 ALS 人群在第一次（中位随访 3 个月）和第二次（中位随访 6 个月）随访时的功能下降与呼吸评估的基线值显著相关（均 p<0.01），但在球部功能障碍患者中仅与基线 PhrenicAmp 相关（均 p<0.05）。回归分析显示，PhrenicAmp（均 p<0.05），而不是 FVC，是 ALS 患者和球部功能障碍患者功能下降的独立预测因子。
基线 PhrenicAmp 是 ALS 功能下降的独立预测因子，无论是否存在球部功能障碍。
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