慢性炎症性脱髓鞘性多神经病变和多灶性运动神经病变的调查和治疗方法的证据基础。

IF 2.3 Q3 CLINICAL NEUROLOGY Neurodegenerative disease management Pub Date : 2022-02-01 Epub Date: 2022-01-10 DOI:10.2217/nmt-2021-0015
Hendrik Stephan Goedee, Yusuf A Rajabally
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引用次数: 0

摘要

慢性炎症性脱髓鞘性多神经病变及其变体和多灶性运动神经病变属于具有复杂免疫功能障碍疾病机制的周围神经疾病谱。了解独特的临床表型和患者之间的异质性对于早期怀疑和订购适当的测试至关重要。这包括对最佳电诊断方案的要求,旨在为相关异常捕获足够的电生理学证据,最终需要进一步扩大诊断设备并正确读取其结果的基于病例的方法。相当大的表型差异,诊断测试中发现的异常的不同组合以及疾病病程和治疗反应的异质性,都导致了及时诊断和最佳治疗成功率的广泛差异。我们的目标是提供有关诊断和管理策略的实用概述和指导,包括陷阱,并对该领域的相关新发展进行总结。
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Evidence base for investigative and therapeutic modalities in chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy.

Chronic inflammatory demyelinating polyneuropathy, its variants and multifocal motor neuropathy belong to a spectrum of peripheral nerve disorders with complex dysimmune disease mechanisms. Awareness of the unique clinical phenotypes but also heterogeneity between patients is vital to arrive at early suspicion and ordering appropriate tests. This includes requirements for optimal electrodiagnostic protocol, aimed to capture sufficient electrophysiologic evidence for relevant abnormalities, a case-based approach on the eventual need to further expand the diagnostic armamentarium and correct reading of their results. Considerable phenotypical variation, diverse combinations of abnormalities found on diagnostic tests and heterogeneity in disease course and treatment response, all contribute to widespread differences in success rates on timely diagnosis and optimal treatment. We aim to provide a practical overview and guidance on relevant diagnostic and management strategies, including pitfalls and present a summary of the relevant novel developments in this field.

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