Conduction slowing, conduction block and temporal dispersion in demyelinating, dysmyelinating and axonal neuropathies: Electrophysiology meets pathology

IF 3.9 3区 医学 Q1 CLINICAL NEUROLOGY Journal of the Peripheral Nervous System Pub Date : 2024-04-10 DOI:10.1111/jns.12625
Antonino Uncini, Tiziana Cavallaro, Gian Maria Fabrizi, Fiore Manganelli, Jean-Michel Vallat
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Abstract

Nerve conduction studies are usually the first diagnostic step in peripheral nerve disorders and their results are the basis for planning further investigations. However, there are some commonplaces in the interpretation of electrodiagnostic findings in peripheral neuropathies that, although useful in the everyday practice, may be misleading: (1) conduction block and abnormal temporal dispersion are distinctive features of acquired demyelinating disorders; (2) hereditary neuropathies are characterized by uniform slowing of conduction velocity; (3) axonal neuropathies are simply diagnosed by reduced amplitude of motor and sensory nerve action potentials with normal or slightly slow conduction velocity. In this review, we reappraise the occurrence of uniform and non-uniform conduction velocity slowing, conduction block and temporal dispersion in demyelinating, dysmyelinating and axonal neuropathies attempting, with a translational approach, a correlation between electrophysiological and pathological features as derived from sensory nerve biopsy in patients and animal models. Additionally, we provide some hints to navigate in this complex field.

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脱髓鞘、髓鞘发育不良和轴索神经病中的传导减慢、传导阻滞和时间弥散:电生理学与病理学的结合
神经传导检查通常是诊断周围神经疾病的第一步,其结果是计划进一步检查的基础。然而,在对周围神经病电诊断结果的解释中存在一些常识,虽然在日常实践中很有用,但可能会产生误导:(1) 传导阻滞和异常时间弥散是获得性脱髓鞘疾病的显著特征;(2) 遗传性神经病的特征是传导速度均匀减慢;(3) 轴索神经病的简单诊断是运动和感觉神经动作电位振幅降低,传导速度正常或稍慢。在这篇综述中,我们重新评估了脱髓鞘、髓鞘发育不良和轴索性神经病中出现的均匀和非均匀传导速度减慢、传导阻滞和时间弥散,并尝试用转化的方法将患者和动物模型感觉神经活检得出的电生理学特征和病理学特征联系起来。此外,我们还提供了一些在这一复杂领域中的导航提示。
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来源期刊
CiteScore
6.10
自引率
7.90%
发文量
45
审稿时长
>12 weeks
期刊介绍: The Journal of the Peripheral Nervous System is the official journal of the Peripheral Nerve Society. Founded in 1996, it is the scientific journal of choice for clinicians, clinical scientists and basic neuroscientists interested in all aspects of biology and clinical research of peripheral nervous system disorders. The Journal of the Peripheral Nervous System is a peer-reviewed journal that publishes high quality articles on cell and molecular biology, genomics, neuropathic pain, clinical research, trials, and unique case reports on inherited and acquired peripheral neuropathies. Original articles are organized according to the topic in one of four specific areas: Mechanisms of Disease, Genetics, Clinical Research, and Clinical Trials. The journal also publishes regular review papers on hot topics and Special Issues on basic, clinical, or assembled research in the field of peripheral nervous system disorders. Authors interested in contributing a review-type article or a Special Issue should contact the Editorial Office to discuss the scope of the proposed article with the Editor-in-Chief.
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