Brain Center UMC Utrecht, Department of Neurology & Neurosurgery, University Medical Centre Utrecht, Utrecht, The Netherlands.
Inflammatory Neuropathy Clinic, Department of Neurology, University Hospitals Birmingham, Birmingham, UK.
Neurodegener Dis Manag. 2022 Feb;12(1):35-47. doi: 10.2217/nmt-2021-0015. Epub 2022 Jan 10.
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.
慢性炎症性脱髓鞘性多发性神经病、其变体和多灶性运动神经病属于周围神经疾病谱，具有复杂的免疫疾病机制。了解独特的临床表型以及患者之间的异质性对于早期怀疑和适当的检测至关重要。这包括对最佳电诊断方案的要求，旨在捕获足够的电生理证据以发现相关的异常，基于病例的方法最终需要进一步扩展诊断工具，并正确解读结果。显著的表型变异、在诊断测试中发现的异常的多种组合以及疾病过程和治疗反应的异质性，都导致了在及时诊断和最佳治疗方面成功率的广泛差异。我们旨在提供相关诊断和管理策略的实用概述和指导，包括陷阱，并总结该领域的相关新进展。
