Amyotrophic lateral sclerosis (ALS) belongs to a group of disorders known as motor neuron diseases. Despite being one of the most devastating diseases known, there is little evidence for diagnosing and managing patients with ALS. Clinical neurophysiologic tests are essential, when no biological marker exists to aid early diagnosis, not only in relation to diagnosis, but also in the development of disease progression, and perhaps, in the future, in measuring patients' response to therapy. The electrophysiological features used in the diagnosis of ALS are based on Awaji-shima consensus recommendations for the application of electrophysiological tests, as applied to the revised El Escorial Criteria. Measurements of axonal excitability through nerve conduction study (ENG) is useful to evaluate axonal degeneration. Electromyography (EMG) recordings with needle examination are essential for confirming lower motor neuron involvement in the initial diagnosis of ALS. EMG abnormalities are frequent and these include fibrillation potentials or positive sharp wave potentials, or both, with fasciculation potentials in resting muscle, and an incomplete interference pattern, with abnormal motor unit potentials. Collateral or terminal nerve sprouting is common in ALS and is frequent large macro-motor unit potentials (MUPs). Motor unit number estimation (MUNE) may be useful in measuring loss of functioning motor units and is an attractive endpoint measure in clinical drug trials in ALS because it directly assesses loss of lower motor neurons and is sensitive to disease progression. Transcortical magnetic stimulation protocols, and cortical excitability may be useful to assess the involvement of upper motor neuron system. In this chapter the advantages, limitations and promise of these various methods are discussed, in order to indicate the direction for further neurophysiological studies in this disorder.

中文翻译：

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ALS的临床神经生理学。

肌萎缩性侧索硬化症（ALS）属于一组称为运动神经元疾病的疾病。尽管是已知的最具破坏性的疾病之一，但几乎没有证据可以诊断和治疗ALS患者。当不存在任何生物标记物来帮助早期诊断时，不仅在诊断方面，而且在疾病进展的发展中，甚至在将来，在测量患者对治疗的反应方面，临床神经生理学检查都是必不可少的。诊断ALS所用的电生理特征是基于Awaji-shima共识性建议，适用于经修订的El Escorial标准的电生理测试应用。通过神经传导研究（ENG）测量轴突兴奋性对评估轴突变性很有用。带有针头检查的肌电图（EMG）记录对于确认下运动神经元参与ALS的初步诊断至关重要。EMG异常很常见，包括颤动电位或正尖波电位，或两者兼有，静息肌有束缚电位，运动模式不完整，运动单位电位异常。侧支或末梢神经发芽在ALS中很常见，并且经常出现大型大型运动单位电位（MUPs）。运动单位数量估计（MUNE）可用于测量功能性运动单位的损失，并且在ALS的临床药物试验中是有吸引力的终点指标，因为它可以直接评估下运动神经元的损失并对疾病进展敏感。经皮磁刺激方案 皮层兴奋性可能对评估上运动神经元系统的参与有用。在本章中，将讨论这些各种方法的优点，局限性和前景，以便为该疾病的进一步神经生理学研究指明方向。