Libonati Laura, Cambieri Chiara, Ceccanti Marco, Moret Federica, Di Giulio Matteo, Palma Eleonora, Inghilleri Maurizio
Department of Human Neurosciences, Rare Neuromuscular Diseases Center, Sapienza University of Rome, Viale Dell'Università, Rome, Italy.
Department of Physiology and Pharmacology, Istituto Pasteur-Fondazione Cenci Bolognetti, University of Rome Sapienza, Rome, Italy.
Front Neurol. 2025 May 7;16:1590950. doi: 10.3389/fneur.2025.1590950. eCollection 2025.
This study investigated differences in muscle twitch force between slow and fast progressors of amyotrophic lateral sclerosis (ALS) to better understand disease heterogeneity and identify potential biomarkers of disease progression.
Forty-four ALS patients were classified as slow or fast progressors based on disease progression rates. Electrophysiological assessments, including compound muscle action potential (CMAP) and muscle force measurements, were conducted. Creatine kinase (CK) levels were also evaluated.
Slow progressors demonstrated significantly higher muscle peak force and area under the curve (AUC) compared to fast progressors, reflecting greater muscle strength and endurance. CK levels were also elevated in slow progressors.
Despite similar CMA values, slow progressors retained greater muscle strength, possibly due to a reduced degeneration of fast-twitch fibers and compensatory axonal sprouting. These adaptations may preserve muscle function and elevate CK levels, suggesting better muscle integrity in slow progressors.
Muscle function profiles and CK levels are promising indicators of ALS progression. These findings could enhance early detection of disease progression and lead to targeted interventions to preserve muscle function. Further research is needed to validate these results and explore the underlying functional mechanisms of disease heterogeneity.
本研究调查了肌萎缩侧索硬化症（ALS）进展缓慢者和进展快速者之间肌肉抽搐力的差异，以更好地理解疾病异质性并确定疾病进展的潜在生物标志物。
根据疾病进展速度将44例ALS患者分为进展缓慢者或进展快速者。进行了包括复合肌肉动作电位（CMAP）和肌肉力量测量在内的电生理评估。还评估了肌酸激酶（CK）水平。
与进展快速者相比，进展缓慢者的肌肉峰值力和曲线下面积（AUC）显著更高，反映出更强的肌肉力量和耐力。进展缓慢者的CK水平也有所升高。
尽管复合肌肉动作电位（CMA）值相似，但进展缓慢者保留了更强的肌肉力量，这可能是由于快肌纤维的退化减少和轴突发芽的代偿作用。这些适应性变化可能维持肌肉功能并提高CK水平，表明进展缓慢者的肌肉完整性更好。
肌肉功能特征和CK水平是ALS进展的有前景的指标。这些发现可能加强对疾病进展的早期检测，并导致采取针对性干预措施来维持肌肉功能。需要进一步研究来验证这些结果并探索疾病异质性的潜在功能机制。
