Helekar Santosh A, Thonhoff Jason, John Blessy S, Nguyen Lisa, Rosenfield David B, Appel Stanley H
Stanley H. Appel Department of Neurology, Houston Methodist Neurological Institute, Houston Methodist Hospital, Houston, TX, 77030, USA.
Weill Cornell Medical College, New York, NY, 10065, USA.
J Neurol. 2022 Oct;269(10):5487-5496. doi: 10.1007/s00415-022-11214-8. Epub 2022 Jun 15.
Patients with amyotrophic lateral sclerosis (ALS) show altered cortical excitability. In this study, we measure modulation of spontaneous motor unit potentials (sMUPs) in hand muscles by multifocal cortical stimulation with a newly developed wearable transcranial rotating permanent magnet stimulator (TRPMS).
We conducted cross-sectional and longitudinal electromyographic assessments in 40 and 20 ALS patients, respectively, of the stimulation-induced peak increase in the count of sMUPs in two hand muscles modulated by unilateral TRPMS stimulation of the primary motor cortex. We measured peak sMUP counts during several short sessions consisting of 10 stimuli over 60 s and 30 s post-stimulation periods. The longitudinal component involved an initial assessment at an early stage of the disease and up to five follow-up assessments at least 3 months apart.
TRPMS stimulation produced no device-related adverse effects. It showed an inverted V-shaped modulation of the peak sMUP counts as a function of ALS functional rating scale revised scores. The ratios of ALS subjects showing peak sMUP count increases between early and intermediate stages (χ2 = 4.086, df = 1, p = 0.043) and intermediate and late stages (χ2 = 4.29, df = 1, p = 0.038) in cross-sectional data were significantly different. Longitudinal assessment also produced a significant (z = 2.31, p = 0.021) result, with all subjects showing a post-initial visit increase in peak sMUP counts.
These results are consistent with delayed onset of upper motor neuronal dysfunction with respect to onset of clinical features. However, the above results need to be confirmed in a larger sample of patients and with multiple lines of evidence.
肌萎缩侧索硬化症（ALS）患者表现出皮质兴奋性改变。在本研究中，我们使用新开发的可穿戴式经颅旋转永磁刺激器（TRPMS），通过多焦点皮质刺激来测量手部肌肉中自发运动单位电位（sMUPs）的调制情况。
我们分别对40例和20例ALS患者进行了横断面和纵向肌电图评估，通过对初级运动皮质进行单侧TRPMS刺激，来观察其对两块手部肌肉中sMUPs计数的刺激诱导峰值增加情况。我们在几个短时间段内测量了sMUPs峰值计数，每个时间段包括60秒内的10次刺激以及刺激后30秒的时间段。纵向评估包括在疾病早期进行的初始评估以及至少相隔3个月的多达五次随访评估。
TRPMS刺激未产生与设备相关的不良反应。它显示出sMUPs峰值计数随ALS功能评定量表修订分数呈倒V形调制。横断面数据中，在疾病早期和中期（χ2 = 4.086，自由度 = 1，p = 0.043）以及中期和晚期（χ² = 4.29，自由度 = 1，p = 0.038）之间，显示sMUPs峰值计数增加的ALS受试者比例存在显著差异。纵向评估也得出了显著结果（z = 2.31，p = 0.021），所有受试者在初次就诊后sMUPs峰值计数均增加。
这些结果与上运动神经元功能障碍相对于临床特征发作的延迟发作一致。然而，上述结果需要在更大样本的患者中并通过多条证据进行证实。
