Department of Neurology, Section of Clinical Neurophysiology, Faculty of Medicine, Gazi University, Ankara, Turkey.
Neuroscience and Neurotechnology Center of Excellence, Ankara, Turkey.
Muscle Nerve. 2024 Dec;70(6):1257-1262. doi: 10.1002/mus.28278. Epub 2024 Oct 11.
INTRODUCTION/AIMS: While amyotrophic lateral sclerosis (ALS) is primarily characterized as a motor system disorder, there is a growing body of evidence indicating sensory involvement. This study aimed to examine the hypothesis that somatosensory processing is impaired in ALS.
Study participants were ALS patients followed at the Neuromuscular Outpatient Unit, as well as healthy volunteers, from March 2021 to July 2023. The Medical Research Council (MRC) sum score was calculated for nine muscle groups bilaterally. The clinical status of patients was evaluated with the ALS Functional Rating Scale-Revised (ALSFRS-R) and the Penn Upper Motor Neuron core. Somatosensory temporal discrimination thresholds (STDTs) were recorded on the medial and lateral parts of both hands. Somatosensory cortex excitability was investigated with the paired somatosensory evoked potentials (SEP) paradigm in a subgroup.
Increased STD values were detected in ALS patients compared to controls in both medial (107.66 ± 35 ms vs. 82.7 ± 32.5 ms, p = .001) and lateral (106.5 ± 34.5 ms vs. 82.9 ± 31.3 ms, p = .002) hands. There were no significant differences in STDTs among ALS patients across four regions (medial and lateral parts of the right and left hands). Amplitude ratios obtained from the paired-pulse SEP paradigm were approximately 1 for all interstimulus intervals (ISIs). STDTs did not show any correlations with motor findings or scales.
Somatosensory processing appears to be compromised among ALS patients. The lack of correlation between impaired STDT and motor findings implies that it is a purely sensory deficit in ALS.
简介/目的：虽然肌萎缩侧索硬化症（ALS）主要表现为运动系统障碍，但越来越多的证据表明其涉及感觉系统。本研究旨在检验假设，即 ALS 患者的躯体感觉处理存在障碍。
研究参与者为 2021 年 3 月至 2023 年 7 月在神经肌肉门诊就诊的 ALS 患者以及健康志愿者。双侧 9 组肌肉的医学研究委员会（MRC）总和评分进行计算。患者的临床状况采用 ALS 功能评定量表修订版（ALSFRS-R）和宾夕法尼亚上运动神经元核心量表进行评估。在亚组中，使用配对体感诱发电位（SEP）范式记录体感时间辨别阈值（STDT）。
与对照组相比，ALS 患者双侧手部的内侧面（107.66 ± 35ms 比 82.7 ± 32.5ms，p = 0.001）和外侧面（106.5 ± 34.5ms 比 82.9 ± 31.3ms，p = 0.002）的 STD 值均升高。在四个区域（右侧和左侧手部的内侧面和外侧面）中，ALS 患者之间的 STD 值无显著差异。在所有刺激间隔（ISIs）下，从成对脉冲 SEP 范式获得的幅度比均接近 1。STDT 与运动发现或量表均无相关性。
ALS 患者的躯体感觉处理似乎受损。STDT 受损与运动发现之间缺乏相关性表明，它是 ALS 中的纯粹感觉缺陷。
