Yu Huiyan, Chen Lu, Zhang Shuo, He Jing, Fan Dongsheng
Department of Neurology, Peking University Third Hospital, Beijing, China.
Department of Neurology, National Center of Gerontology, Beijing Hospital, Beijing, China.
Front Neurol. 2021 Feb 11;12:574919. doi: 10.3389/fneur.2021.574919. eCollection 2021.
To assess the prognostic value of the decrement in compound muscle action potential amplitude within 12 months of symptom onset (CMAP-12 amplitude) for the survival of patients with amyotrophic lateral sclerosis (ALS). Patients were stratified into 4 groups according to the decrement of the CMAP-12 amplitudes: normal (≥the lower limit of normal, LLN), mild (<LLN but ≥50% of LLN), moderate (<50% but ≥30% of LLN) and severe (<30% of LLN). All patients were followed up every 3 months. Survival was analyzed using the Kaplan-Meier method and Cox proportional hazards regression. A total of 149 patients were included in the analysis [90 males (60.4%); mean age at onset, 50.7 years]. The decrement of CMAP-12 amplitudes was normal in 24.2% of patients, mild in 22.1%, moderate in 15.4% and severe in 38.3%. Kaplan-Meier analysis showed there was a significant difference in the overall survival across the 4 groups ( < 0.05). Further pairwise comparisons identified significant differences in survival between the normal . the moderate group ( < 0.05) and the normal . the severe group ( < 0.01). There was a significant inverse correlation between the CMAP-12 amplitude and overall survival. Compared to that in the normal group, survival in the moderately and severely decreased groups was significantly shorter (HR 3.394, 95% CI 1.292-8.917, = 0.013; and HR 4.732, 95% CI 2.032-11.017; = 0.000, respectively). Our results suggest that CMAP-12 amplitude could be a prognostic indicator of disease progression in ALS. More importantly, our findings provide clinical evidence for the viewpoint that early axonal dysfunction of the peripheral nervous system accelerates disease progression of ALS.
评估肌萎缩侧索硬化症（ALS）患者症状出现后12个月内复合肌肉动作电位幅度下降（CMAP-12幅度）对患者生存的预后价值。根据CMAP-12幅度下降情况将患者分为4组：正常（≥正常下限，LLN）、轻度（<LLN但≥LLN的50%）、中度（<50%但≥LLN的30%）和重度（<LLN的30%）。所有患者每3个月随访一次。采用Kaplan-Meier法和Cox比例风险回归分析生存情况。共有149例患者纳入分析[90例男性（60.4%）；发病时平均年龄50.7岁]。24.2%的患者CMAP-12幅度下降正常，22.1%为轻度，15.4%为中度，38.3%为重度。Kaplan-Meier分析显示4组患者的总生存期存在显著差异（<0.05）。进一步的两两比较发现，正常组与中度组（<0.05）以及正常组与重度组（<0.01）之间的生存存在显著差异。CMAP-12幅度与总生存期呈显著负相关。与正常组相比，中度和重度下降组的生存期显著缩短（HR 3.394，95%CI 1.292 - 8.917，= 0.013；HR 4.732，95%CI 2.032 - 11.017；= 0.000）。我们的结果表明，CMAP-12幅度可能是ALS疾病进展的预后指标。更重要的是，我们的研究结果为外周神经系统早期轴突功能障碍加速ALS疾病进展这一观点提供了临床证据。
