Department of Neurology and Clinical Neurophysiology, Amsterdam Neuroscience, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands.
Department of Neurology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
Brain. 2020 Dec 5;143(11):3214-3224. doi: 10.1093/brain/awaa265.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) consists of a spectrum of autoimmune diseases of the peripheral nerves, causing weakness and sensory symptoms. Diagnosis often is challenging, because of the heterogeneous presentation and both mis- and underdiagnosis are common. Nerve conduction study (NCS) abnormalities suggestive of demyelination are mandatory to fulfil the diagnostic criteria. On the one hand, performance and interpretation of NCS can be difficult and none of these demyelinating findings are specific for CIDP. On the other hand, not all patients will be detected despite the relatively high sensitivity of NCS abnormalities. The electrodiagnostic criteria can be supplemented with additional diagnostic tests such as CSF examination, MRI, nerve biopsy, and somatosensory evoked potentials. However, the evidence for each of these additional diagnostic tests is limited. Studies are often small without the use of a clinically relevant control group. None of the findings are specific for CIDP, meaning that the results of the diagnostic tests should be carefully interpreted. In this update we will discuss the pitfalls in diagnosing CIDP and the value of newly introduced diagnostic tests such as nerve ultrasound and testing for autoantibodies, which are not yet part of the guidelines.
慢性炎症性脱髓鞘性多发性神经病（CIDP）是一组自身免疫性疾病，影响外周神经系统，导致虚弱和感觉症状。由于表现形式多样，且误诊和漏诊都很常见，因此诊断常常具有挑战性。神经传导研究（NCS）异常提示脱髓鞘是符合诊断标准所必需的。一方面，NCS 的性能和解释可能具有挑战性，而且这些脱髓鞘发现都没有特异性。另一方面，尽管 NCS 异常具有相对较高的敏感性，但并非所有患者都能被检测到。电诊断标准可以通过其他诊断测试来补充，如脑脊液检查、MRI、神经活检和体感诱发电位。然而，这些附加诊断测试的证据都有限。这些研究通常规模较小，且没有使用临床相关的对照组。这些发现都没有特异性，这意味着诊断测试的结果应该仔细解释。在本次更新中，我们将讨论诊断 CIDP 的陷阱，以及新引入的诊断测试（如神经超声和自身抗体检测）的价值，这些测试目前尚未纳入指南。
