Abstract

Objective

In amyotrophic lateral sclerosis (ALS), early recognition of nocturnal hypoventilation (NH) is essential to start noninvasive ventilation (NIV), but nocturnal transcutaneous PCO₂ (PtcCO₂) is difficult to monitor. Usefulness of respiratory and muscular function test in the prediction of NH has been explored without distinguishing among ALS phenotypes. We evaluated cross-sectional relationships between functional tests and nocturnal PCO₂, and the best predictors of NH, separately in patients with spinal and bulbar onset of ALS. Methods: ALS patients candidate to NIV were recruited. Diurnal respiratory and muscular function tests and nocturnal polysomnography with PtcCO₂ monitoring were performed. NH was defined as peak PtcCO₂ >49 mm Hg. Results: Thirty-six patients with spinal and 11 with bulbar onset ALS were included. Nocturnal oxygen saturation and PtcCO₂, and proportion of subjects with NH were similar in each group (spinal: 50%; bulbar: 45.5%). Significant differences between groups were found in forced vital capacity (p = 0.03), maximal inspiratory pressure (p = 0.01) and sniff nasal inspiratory pressure (SNIP) (p = 0.007), but not in diurnal arterial blood gases. In the spinal group, SNIP and Base Excess (BE) independently predicted nocturnal PtcCO₂ (R² 0.59, p < 0.0001). In the bulbar group only SNIP was correlated to PtcCO₂, but it varied little in relationship to PtcCO₂ changes. Conclusions: Respiratory and muscle function parameters are differently related to NH in ALS patients with spinal and bulbar presentation. SNIP and BE may be helpful to reveal NH in spinal patients, while in bulbar patients no respiratory or muscle function tests may reliably predict NH.

中文翻译：

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肌萎缩侧索硬化症夜间通气不足预测因子的异质性

摘要

客观的

在肌萎缩侧索硬化症 (ALS) 中，早期识别夜间低通气 (NH) 对于开始无创通气 (NIV) 至关重要，但夜间经皮 PCO ₂ (PtcCO ₂ ) 难以监测。已经探索了呼吸和肌肉功能测试在预测 NH 中的有用性，但未区分 ALS 表型。_{我们评估了功能测试和夜间 PCO 2}之间的横截面关系，以及 NH 的最佳预测因子，分别在脊髓和延髓发病的 ALS 患者中进行。方法: 招募 NIV 候选 ALS 患者。使用 PtcCO _{2进行的昼夜呼吸和肌肉功能测试以及夜间多导睡眠图}进行了监测。NH 定义为峰值PtcCO ₂ >49 mm Hg。结果：纳入了 36 名脊髓型肌萎缩侧索硬化患者和 11 名延髓型肌萎缩侧索硬化患者。夜间血氧饱和度和 PtcCO ₂以及 NH 受试者的比例在各组中相似（脊柱：50%；延髓：45.5%）。组间在用力肺活量 ( p  = 0.03)、最大吸气压力 ( p  = 0.01) 和嗅鼻吸气压力 (SNIP) ( p  = 0.007) 方面存在显着差异，但在昼夜动脉血气方面无显着差异。在脊髓组中，SNIP 和 Base Excess (BE) 独立预测夜间 PtcCO ₂ (R ² 0.59, p < 0.0001)。在延髓组中，只有 SNIP 与 PtcCO ₂相关，但与 PtcCO ₂变化的关系变化不大。结论：在脊柱和延髓表现的 ALS 患者中，呼吸和肌肉功能参数与 NH 的相关性不同。SNIP 和 BE 可能有助于揭示脊柱患者的 NH，而在延髓患者中，没有呼吸或肌肉功能测试可以可靠地预测 NH。