Neurophysiological aspects of the completely locked-in syndrome in patients with advanced amyotrophic lateral sclerosis
Neurophysiological aspects of the completely locked-in syndrome in patients with advanced amyotrophic lateral sclerosis
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DOI：
10.1016/j.clinph.2021.01.013
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发表时间：
2021-03-17
影响因子：
4.7
通讯作者：
Chaudhary, Ujwal
中科院分区：
文献类型：
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作者：
Khalili-Ardali, Majid;Wu, Shizhe;Chaudhary, Ujwal
目的：完全锁定综合征（CLIS）的肌萎缩侧索硬化症（ALS）患者无法表达自己，其意识和意识状态难以评估。由于完全麻痹包括眼部肌肉麻痹，对知觉和心理生理状态的任何评估只能在具有神经生理学记录的被动实验范式中实施。方法：在 CLIS 中的四名患者在多项实验中进行了研究，包括静息状态、视觉刺激（睁眼与闭眼）、听觉刺激（改良的局部-全局范式）、体感刺激（正中神经的电刺激）和睡眠期间。结果：所有患者都表现出神经生理学指标的改变，但独特和患者之间无法找到共同模式。然而，脑电图 (EEG) 减慢和 α 波活动减弱或缺失在所有患者中都很常见。在四名患者中的两名患者中，出现了 4 Hz 的缓慢主频率，并且所有通道的 EEG 均同步。在另外两名患者中，脑电图的减慢似乎不太同步。所有患者睁眼和闭眼之间的脑电图存在显着差异。白天的主导慢频率在慢波睡眠（据称是睡眠第 3 阶段）期间发生变化，甚至变为低于 2 Hz 的更慢频率。与健康受试者相比，体感诱发电位 (SEP) 缺失或显着改变，听觉诱发电位 (AEP) 也类似。 结论：结果的异质性强调了这样一个事实：没有单一的神经生理指标可用于评估 CLIS 中无反应的 ALS 患者的心理生理状态。这一警告对于认知过程的评估也可能有效。意义：大多数关于ALS患者神经生理学的研究都集中在疾病的早期阶段，而对于患者完全瘫痪、没有任何沟通手段（即CLIS）的晚期阶段的研究很少。这项研究提供了这些患者不同神经生理学方面的定量指标。(c) 2021 年国际临床神经生理学联合会。由 Elsevier B.V. 出版。保留所有权利。
Objective: Amyotrophic lateral sclerosis (ALS) patients in completely locked-in syndrome (CLIS) are incapable of expressing themselves, and their state of consciousness and awareness is difficult to evaluate. Due to the complete paralysis included paralysis of eye muscles, any assessment of the perceptual and psychophysiological state can only be implemented in passive experimental paradigms with neurophysiological recordings.Methods: Four patients in CLIS were investigated in several experiments including resting state, visual stimulation (eyes open vs eyes closed), auditory stimulation (modified local-global paradigm), somatosensory stimulation (electrical stimulation of the median nerve), and during sleep.Results: All patients showed altered neurophysiological metrics, but a unique and common pattern could not be found between patients. However, slowing of the electroencephalography (EEG) and attenuation or absence of alpha wave activity was common in all patients. In two of the four patients, a slow dominant frequency emerged at 4 Hz with synchronized EEG at all channels. In the other two patients slowing of EEG appears less synchronized. EEGs between eyes open and eyes closed were significantly different in all patients. The dominant slow frequency during the day changes during slow-wave sleep (supposedly sleep stage 3) to even slower frequencies below 2 Hz. Somatosensory evoked potentials (SEPs) were absent or significantly altered in comparison to healthy subjects, similarly for auditory evoked potentials (AEPs).Conclusions: The heterogeneity of the results underscores the fact that no single neurophysiological index is available to assess psychophysiological states in unresponsive ALS patients in CLIS. This caveat may also be valid for the assessment of cognitive processes; a functioning BCI can be the solution.Significance: Most of the studies of the neurophysiology of ALS patients focused on the early stage of the disease, and there are very few studies on the late stage when patients are completely paralyzed with no means of communication (i.e., CLIS). This study provides quantitative metrics of different neurophysiological aspects of these patients.(c) 2021 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.