The puzzling case of hyperexcitability in amyotrophic lateral sclerosis.
The puzzling case of hyperexcitability in amyotrophic lateral sclerosis.
复制标题
DOI：
10.3988/jcn.2013.9.2.65
复制
发表时间：
2013-04
期刊：
影响因子：
--
通讯作者：
Kiernan MC
中科院分区：
文献类型：
--
作者：
Bae JS;Simon NG;Menon P;Vucic S;Kiernan MC
肌萎缩侧索硬化症（ALS）的过度兴奋性的发展是一个众所周知的现象。尽管存在争议的潜在机制，皮质过度兴奋似乎是密切相关的兴奋性corticomotoneurons和抑制性interneurons之间的相互作用。过度兴奋不是一种静态现象，而是在时空方面显示出一种进展模式。皮质过度兴奋可能作为一个触发器的发展，前角细胞变性通过一个'死亡前进'的过程。过度兴奋似乎在疾病的早期阶段发展，并在疾病的晚期阶段逐渐消失，与皮质运动神经元通路的破坏有关。因此，对这些独特过程的更精确解释可能会提供关于ALS及其临床特征的病理生理学的新见解。最近开发的技术，如阈值跟踪经颅磁刺激和自动神经兴奋性测试提供了一些线索，潜在的病理生理过程与过度兴奋。此外，这些新技术使临床医生能够使用过度兴奋的特异性发现作为有用的诊断生物标志物，从而能够澄清各种ALS模拟综合征，并预测家族性ALS症状前携带者的疾病发展。在外周神经的神经兴奋性测试方面，持续Na+电导的增加已被确定为ALS中外周过度兴奋的主要决定因素，与ALS中的存活率呈负相关。因此，本综述将主要集中在ALS的过度兴奋的令人困惑的理论和总结临床和病理生理学的影响，为当前和未来的ALS研究。
The development of hyperexcitability in amyotrophic lateral sclerosis (ALS) is a well-known phenomenon. Despite controversy as to the underlying mechanisms, cortical hyperexcitability appears to be closely related to the interplay between excitatory corticomotoneurons and inhibitory interneurons. Hyperexcitability is not a static phenomenon but rather shows a pattern of progression in a spatiotemporal aspect. Cortical hyperexcitability may serve as a trigger to the development of anterior horn cell degeneration through a 'dying forward' process. Hyperexcitability appears to develop during the early disease stages and gradually disappears in the advanced stages of the disease, linked to the destruction of corticomotorneuronal pathways. As such, a more precise interpretation of these unique processes may provide new insight regarding the pathophysiology of ALS and its clinical features. Recently developed technologies such as threshold tracking transcranial magnetic stimulation and automated nerve excitability tests have provided some clues about underlying pathophysiological processes linked to hyperexcitability. Additionally, these novel techniques have enabled clinicians to use the specific finding of hyperexcitability as a useful diagnostic biomarker, enabling clarification of various ALS-mimic syndromes, and the prediction of disease development in pre-symptomatic carriers of familial ALS. In terms of nerve excitability tests for peripheral nerves, an increase in persistent Na+ conductances has been identified as a major determinant of peripheral hyperexcitability in ALS, inversely correlated with the survival in ALS. As such, the present Review will focus primarily on the puzzling theory of hyperexcitability in ALS and summarize clinical and pathophysiological implications for current and future ALS research.
登录
查看更多内容
影响因子：
2.7
作者：
Eisen, A
通讯作者：
Eisen, A
影响因子：
3.4
作者：
CHOU, SM;NORRIS, FH
通讯作者：
NORRIS, FH
影响因子：
14.5
作者：
Burrell, James R.;Kiernan, Matthew C.;Hodges, John R.
通讯作者：
Hodges, John R.
影响因子：
9.9
作者：
Caramia, MD;Palmieri, MG;Bernardi, G
通讯作者：
Bernardi, G
影响因子：
4.7
作者：
Cheah, Benjamin C.;Lin, Cindy S. Y.;Kiernan, Matthew C.
通讯作者：
Kiernan, Matthew C.