<br>慢性炎症性脱髓鞘性多发性神经根神经病的诊断挑战,Brain

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慢性炎症性脱髓鞘性多发性神经根神经病的诊断挑战
Brain ( IF 10.6 ) Pub Date : 2020-11-06 , DOI: 10.1093/brain/awaa265
Filip Eftimov ₁ , Ilse M Lucke ₁ , Luis A Querol _{2,

3} , Yusuf A Rajabally ₄ , Camiel Verhamme ₁

Affiliation

慢性炎症性脱髓鞘性多发性神经根神经病（CIDP）由一系列周围神经自身免疫性疾病组成，可导致无力和感觉症状。由于表现形式多样，误诊和漏诊也很常见，因此诊断往往具有挑战性。提示脱髓鞘的神经传导研究（NCS）异常必须满足诊断标准。一方面，NCS 的表现和解释可能很困难，而且这些脱髓鞘检查结果都不是 CIDP 特有的。另一方面，尽管 NCS 异常的敏感性相对较高，但并非所有患者都会被检测到。电诊断标准可以补充额外的诊断测试，例如脑脊液检查、MRI、神经活检和体感诱发电位。然而，这些额外诊断测试的证据都是有限的。研究通常规模较小，没有使用临床相关的对照组。所有发现均不是 CIDP 特有的，这意味着诊断测试的结果应仔细解释。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（例如神经超声和自身抗体测试）的价值，这些测试尚未成为指南的一部分。

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Diagnostic challenges in chronic inflammatory demyelinating polyradiculoneuropathy

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.

更新日期：2020-12-06

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