Effects of motor cortical and peripheral axonal hyperexcitability on survival in amyotrophic lateral sclerosis

肌萎缩侧索硬化 磁刺激 神经科学 外围设备 沉默期 运动神经元 运动皮层 上运动神经元 医学 刺激 心理学 疾病 内科学 脊髓
作者
Ryo Otani,Kazumoto Shibuya,Yoichi Suzuki,Tomoki Suichi,Marie Morooka,Yuya Aotsuka,Moeko Ogushi,Satoshi Kuwabara
出处
期刊:Journal of Neurology, Neurosurgery, and Psychiatry [BMJ]
卷期号:95 (8): 730-736 被引量:3
标识
DOI:10.1136/jnnp-2023-333039
摘要

Background Increased ‘cortical’ and ‘peripheral’ excitability are reportedly associated with shorter survival in amyotrophic lateral sclerosis (ALS) patients, suggesting that hyperexcitability contributes to motor neuron death. However, whether upper or lower motor function has a greater impact on survival is unclear. We aimed to investigate the component that strongly impacts the prognosis of ALS. Methods A total of 103 consecutive patients with ALS who underwent cortical (threshold tracking transcranial magnetic stimulation (TMS)) and motor nerve excitability tests were included. Motor cortical excitability was evaluated using short-interval intracortical inhibition (SICI) during TMS. Motor axonal excitability was assessed using the strength-duration time constant (SDTC). Survival time was defined as the time from examination to death or tracheostomy. Results Compared with healthy subjects, patients with ALS had lower SICI and longer SDTC (p<0.05), indicating increased excitability of cortical motor neurons and motor axons. According to the SICI and SDTC findings, patients were divided into the following four groups: ‘cortical high and peripheral high (high-high)’, ‘high-low’, ‘low-high’ and ‘low-low’ groups. In Kaplan-Meier curves, the ‘high-high’ and ‘low-high’ groups showed significantly shorter survival than the other groups. Multivariate analysis revealed that increased cortical (HR=5.3, p<0.05) and peripheral (HR=20.0, p<0.001) excitability were significantly associated with shorter survival. Conclusions In patients with ALS, both motor cortical and peripheral hyperexcitability independently affected survival time, with peripheral hyperexcitability having a greater impact on shorter survival. The modulation of neuronal/axonal excitability is a potential therapeutic target for ALS.
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