Division of Neurology, Department of Medicine, Faculty of Medicine and Health Sciences, Stellenbosch University, Cape Town, South Africa.
School of Economics and Business Sciences, University of the Witwatersrand, Johannesburg, South Africa.
Respir Care. 2022 May;67(5):553-561. doi: 10.4187/respcare.09452.
The Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) is widely employed in assessing functional decline in individuals with amyotrophic lateral sclerosis (ALS). A limitation of the scale is that item 12 does not directly evaluate worsening respiratory failure in ALS but rather the management thereof as a surrogate marker. We propose an alternative scale to assess respiratory function in ALS individuals who do not use noninvasive ventilation (NIV).
85 participants were included in the study. ALSFRS-R scores were calculated and FVC measured at each clinic visit. Additional questions were asked regarding the presence of nocturnal hypoventilation symptoms, including (1) early-morning headaches, (2) excessive daytime somnolence, (3) poor concentration, and (4) decrease in appetite. A nocturnal hypoventilation item was developed using these questions in participants not using NIV. Internal consistency and validity were calculated using the nocturnal hypoventilation item as substitute for the existing item 12. The ALSFRS-R was modified by adding the alternative item 12 and named ALSFRS-Revised Modified (ALSFRS-RM).
The ALSFRS-RM has a strong internal consistency and validity, which was calculated using Cronbach alpha and factor analysis. A Spearman correlation of 0.34 was calculated between the measured FVC and the nocturnal hypoventilation item score. In addition, a nocturnal hypoventilation item score of ≤ 3 corresponds to an FVC of ≤ 65%, with the upper 95% CI < 80%.
Our results suggest that the addition of an alternative item 12 to the existing ALSFRS-R may be a viable option for use in individuals not receiving ventilatory support. The new nocturnal hypoventilation item may also be a reliable indicator of respiratory decline that may remove the need for FVC measurement prior to introducing NIV.
修订后的肌萎缩侧索硬化功能评定量表（ALSFRS-R）广泛用于评估肌萎缩侧索硬化症（ALS）患者的功能下降。该量表的一个局限性在于，第 12 项并非直接评估 ALS 患者呼吸衰竭恶化情况，而是将其作为替代标志物来评估呼吸衰竭的管理情况。我们提出了一种替代量表，用于评估未使用无创通气（NIV）的 ALS 患者的呼吸功能。
本研究纳入了 85 名参与者。在每次就诊时计算 ALSFRS-R 评分并测量 FVC。还询问了有关夜间低通气症状存在情况的其他问题，包括（1）清晨头痛，（2）白天过度嗜睡，（3）注意力不集中，以及（4）食欲下降。在未使用 NIV 的参与者中，使用这些问题开发了一个夜间低通气项目。使用夜间低通气项目作为替代第 12 项的内部一致性和有效性进行了计算。通过添加替代项目 12 对 ALSFRS-R 进行了修改，命名为改良后的 ALSFRS-R（ALSFRS-RM）。
ALSFRS-RM 具有很强的内部一致性和有效性，这是通过 Cronbach α 和因子分析来计算的。通过测量的 FVC 和夜间低通气项目得分之间的 Spearman 相关系数为 0.34。此外，夜间低通气项目得分≤3 对应于 FVC≤65%，其上限 95%CI<80%。
我们的研究结果表明，在未接受通气支持的患者中，在现有的 ALSFRS-R 中添加替代项目 12 可能是一种可行的选择。新的夜间低通气项目也可能是呼吸功能下降的可靠指标，这可能在引入 NIV 之前无需进行 FVC 测量。
