Terao Shin-Ichi, Nosaki Yasunobu, Murao Atsunori, Torii Ryota, Ogawa Nanayo, Miura Naofumi, Sasaki Yousuke, Sobue Gen
Neurology, Mitaki General Hospital, Yokkaichi, Mie, Japan.
Neurology, Toyohashi Municipal Hospital, Toyohashi, Aichi, Japan.
BMJ Neurol Open. 2024 Dec 18;6(2):e000829. doi: 10.1136/bmjno-2024-000829. eCollection 2024.
The objective of this study is to identify factors influencing progression of respiratory decline from the onset of neurological symptoms to respiratory failure in patients with amyotrophic lateral sclerosis (ALS).
In 100 patients with sporadic ALS, %vital capacity (%VC) was continuously measured from the first visit to the respiratory endpoint (REP). Cox proportional hazards model identified factors influencing the duration from onset of ALS to REP (Onset-REP). We performed Kaplan-Meier survival curve analysis for onset-REP according to identified factors.
Onset sites were the upper limb (U-ALS), lower limb (L-ALS), bulbar paralysis (B-ALS) and respiratory paralysis (R-ALS) in 37, 19, 32 and 12 patients, respectively. Duration from the onset of ALS to the onset of respiratory symptoms (Onset-Rp) and REP (Onset-REP) was 16.1 (SD 12.1) and 24.9 months (SD 14.6), respectively. Multivariate analysis revealed that age at onset, site of onset, Onset-Rp and %VC decline rate significantly influenced Onset-REP duration. Elderly patients had a significantly shorter Onset-REP duration. Onset-REP duration did not significantly differ between patients with U-ALS and L-ALS, but was longer in these patients than in those with B-ALS and R-ALS. Onset-REP duration was positively associated with Onset-Rp duration. The average monthly %VC decline rate was -5.6% (SD 3.3). Age at onset, onset site and Onset-Rp duration significantly influenced the %VC decline rate.
Our findings revealed strong and independent patient-specific factors that influence the Onset-REP duration and the %VC decline rate in patients with ALS. These could inform future clinical trials and interventions considering the respiratory function and natural history of patients with ALS.
本研究的目的是确定影响肌萎缩侧索硬化症（ALS）患者从神经症状发作到呼吸衰竭过程中呼吸功能衰退进展的因素。
对100例散发性ALS患者，从首次就诊至呼吸终点（REP）连续测量肺活量百分比（%VC）。Cox比例风险模型确定影响从ALS发作到REP（发作-REP）持续时间的因素。我们根据确定的因素对发作-REP进行了Kaplan-Meier生存曲线分析。
首发部位分别为上肢（U-ALS）、下肢（L-ALS）、延髓麻痹（B-ALS）和呼吸麻痹（R-ALS）的患者有37例、19例、32例和12例。从ALS发作到呼吸症状发作（发作-Rp）和REP（发作-REP）的持续时间分别为16.1个月（标准差12.1）和24.9个月（标准差14.6）。多因素分析显示，发病年龄、首发部位、发作-Rp和%VC下降率显著影响发作-REP持续时间。老年患者的发作-REP持续时间明显较短。U-ALS和L-ALS患者的发作-REP持续时间无显著差异，但这些患者的发作-REP持续时间比B-ALS和R-ALS患者长。发作-REP持续时间与发作-Rp持续时间呈正相关。平均每月%VC下降率为-5.6%（标准差3.3）。发病年龄、首发部位和发作-Rp持续时间显著影响%VC下降率。
我们的研究结果揭示了影响ALS患者发作-REP持续时间和%VC下降率的强大且独立的患者特异性因素。这些因素可为未来考虑ALS患者呼吸功能和自然病史的临床试验及干预提供参考。
