Institute of Physiology, Institute of Molecular Medicine, Faculty of Medicine, University of Lisbon, Lisbon, Portugal.
Neurodegener Dis. 2017;17(6):323-329. doi: 10.1159/000480638. Epub 2017 Oct 31.
BACKGROUND/AIMS: The global perception of the health status (HS) of amyotrophic lateral sclerosis (ALS) patients before the initial diagnosis has not been addressed previously.
We recorded the following at the first visit, before diagnostic information: (1) visual analog scale (VAS) of the EQ-5D; (2) the revised ALS functional rating scale (ALSFRS- R), bulbar (ALSFRSb), upper limb (ALSFRSul), lower limb (ALSFRSll), and respiratory (RofALSFRS-R) subscores; and (3) forced and slow vital capacities. Correlations were tested by the Pearson correlation test. Variables were compared between groups defined by the VAS median value. p < 0.05 was considered significant.
Of the 156 patients included in the study (91 spinal-onset, 49 bulbar-onset, 16 axial/respiratory-onset; 95 men; mean onset age 63.9 ± 13 years; mean disease duration 18.4 ± 26.5 months), HS VAS was significantly lower in spinal-onset patients (p = 0.047), and particularly in spinal-onset women (p = 0.027). Disease duration had no influence. HS VAS was moderately correlated with ALSFRS, ALSFRSul and ALSFRSll (0.4 < r < 0.5, p < 0.01), weakly correlated with RofALSFRS-R in the whole population (r = 0.171, p < 0.05), and not correlated with ALSFRSb or the respiratory tests. ALSFRSb was similar between groups defined by the HS VAS median value, but the other scores were significantly lower for poorer HS values.
HS before diagnosis is mostly dependent on the perception of upper and lower limb function. A tool tailored to evaluate HS in bulbar-onset patients should be developed.
背景/目的：之前尚未探讨在初始诊断之前，全球对肌萎缩侧索硬化症（ALS）患者健康状况（HS）的认知。
我们在首次就诊时记录了以下内容，包括诊断信息之前：（1） EQ-5D 视觉模拟量表（VAS）；（2）修订后的 ALS 功能评定量表（ALSFRS-R），球部（ALSFRSb），上肢（ALSFRSul），下肢（ALSFRSll）和呼吸（RofALSFRS-R）子评分；以及（3）用力肺活量和缓慢肺活量。采用 Pearson 相关检验测试相关性。根据 VAS 中位数将变量与组间进行比较。p<0.05 被认为具有统计学意义。
在纳入研究的 156 例患者中（91 例脊髓型，49 例球部型，16 例轴性/呼吸型；95 例男性；平均发病年龄 63.9±13 岁；平均病程 18.4±26.5 个月），脊髓型患者的 HS VAS 明显较低（p=0.047），尤其是脊髓型女性患者（p=0.027）。病程没有影响。HS VAS 与 ALSFRS、ALSFRSul 和 ALSFRSll 呈中度相关（0.4<r<0.5，p<0.01），与整个人群的 RofALSFRS-R 呈弱相关（r=0.171，p<0.05），与 ALSFRSb 或呼吸测试无关。根据 HS VAS 中位数定义的组之间的 ALSFRSb 相似，但其他评分对于较差的 HS 值则明显较低。
诊断前的 HS 主要取决于上肢和下肢功能的感知。应该开发专门用于评估球部型患者 HS 的工具。
