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多发性神经病的神经传导研究:实用生理学与异常模式

Nerve conduction studies in polyneuropathy: practical physiology and patterns of abnormality.

作者信息

Franssen Hessel, van den Bergh Peter Y K

机构信息

Department of Neurology, Neuromuscular Research Group, Rudolf Magnus Institute of Neuroscience, University Medical Center Utrecht, The Netherlands.

出版信息

Acta Neurol Belg. 2006 Jun;106(2):73-81.

Abstract

Nerve conduction studies are an essential part of the work-up of peripheral neuropathies. Many neuropathic syndromes can be suspected on clinical grounds, but optimal use of nerve conduction study techniques (in combination with needle electromyography) allows diagnostic classification and is therefore crucial to understanding and separation of neuropathies. Multifocal motor neuropathy, for example, may clinically present as ALS. Detection of evidence of demyelination (conduction blocks) leads to the correct diagnosis and to proper treatment. Nerve conduction studies provide essential information on (1) the spatial pattern of neuropathy, (2) the pattern of abnormalities distinguishing between primarily axonal and demyelinating pathology, and (3) the severity of neuropathic damage. This information is very comprehensive since many nerves and long segments of individual nerves can be sampled. Moreover the information is extremely detailed to the extent that the cellular pathology of a patient's neuropathy is usually defined best by physiological testing rather than by biopsy. Neuropathies can be generalized, focal, or multifocal; they can be symmetric or asymmetric; they can be distally predominant or proximal and distal. Primarily axonal neuropathies mainly affect sensory nerve and compound muscle action potential amplitudes, whereas demyelinating neuropathies lead to slowing of nerve conductions, and to increased temporal dispersion or conduction block. Usually, the pattern of demyelination allows to distinguish hereditary (uniform demyelination) from acquired (segmental demyelination) neuropathies. Electrodiagnostic criteria for primary demyelination are helpful to identify acquired demyelinating neuropathies.

摘要

神经传导研究是周围神经病检查的重要组成部分。许多神经病变综合征可基于临床症状进行怀疑,但最佳运用神经传导研究技术(结合针极肌电图)可实现诊断分类,因此对于理解和区分神经病至关重要。例如,多灶性运动神经病在临床上可能表现为肌萎缩侧索硬化症。检测到脱髓鞘证据(传导阻滞)可得出正确诊断并进行恰当治疗。神经传导研究提供了关于以下方面的重要信息:(1)神经病的空间分布模式;(2)区分主要为轴索性和脱髓鞘性病变的异常模式;(3)神经病变损伤的严重程度。由于可以对许多神经以及单个神经的长节段进行采样,该信息非常全面。此外,该信息极其详细,以至于患者神经病的细胞病理学通常通过生理测试而非活检来最佳定义。神经病可以是全身性、局灶性或多灶性的;可以是对称或不对称的;可以以远端为主或同时累及近端和远端。主要的轴索性神经病主要影响感觉神经和复合肌肉动作电位的幅度,而脱髓鞘性神经病会导致神经传导减慢,并增加时间离散或传导阻滞。通常,脱髓鞘模式有助于区分遗传性(均匀脱髓鞘)和获得性(节段性脱髓鞘)神经病。原发性脱髓鞘的电诊断标准有助于识别获得性脱髓鞘性神经病。

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