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慢性炎症性脱髓鞘性多发性神经根神经病:塞尔维亚临床实践中的诊断问题。

Chronic inflammatory demyelinating polyradiculoneuropathy: Diagnostic problems in clinical practice in Serbia.

机构信息

University Clinical Center of Serbia, Neurology Clinic, Belgrade, Serbia.

Faculty of Medicine, University of Belgrade, Belgrade, Serbia.

出版信息

J Peripher Nerv Syst. 2023 Jun;28(2):226-236. doi: 10.1111/jns.12537. Epub 2023 Feb 14.

DOI:10.1111/jns.12537
PMID:36738239
Abstract

Making diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is challenging since it can mimic a multitude of disorders, and is misdiagnosed in at least 50% of cases. We sought to determine the frequency of CIDP misdiagnosis in clinical practice in Serbia, to uncover CIDP mimics, and to identify factors that may aid in CIDP diagnosis. Our longitudinal retrospective cohort study included 86 eligible adult patients referred to the Neurology Clinic, University Clinical Centre of Serbia, with a diagnosis of CIDP. We also included 15 patients referred to us with different diagnoses that ended up having CIDP as their final diagnosis. Exactly half of patients referred as CIDP failed to meet the established diagnostic criteria (non-CIDP) and were given an alternative diagnosis at the first hospitalization. At the 1-year follow-up, the diagnosis was further revised in four subjects. Confirmed CIDP patients usually had their initial diagnosis based on the nerve conduction studies (NCS), a typical presentation with symmetrical involvement of all four limbs, as well as higher frequencies of elevated protein levels and albuminocytologic dissociation in the cerebrospinal fluid (CSF). CIDP patients also responded better to immune therapy. We found that 52% of the patients initially referred to our Clinic as CIDP were given other diagnoses after a 1-year follow-up. Out of all CIDP cases, 27% had been unrecognized prior to referral to our Center. Utilization of clear and objective indicators - conclusive NCS, improvement on therapy, and elevated CSF proteins may provide greater certainty in diagnosing CIDP.

摘要

诊断慢性炎症性脱髓鞘性多发性神经病(CIDP)具有挑战性,因为它可能模仿多种疾病,并且至少有 50%的病例被误诊。我们旨在确定 CIDP 在塞尔维亚临床实践中的误诊频率,揭示 CIDP 的模拟病症,并确定可能有助于 CIDP 诊断的因素。我们的纵向回顾性队列研究包括 86 名符合条件的成年患者,他们因 CIDP 被转诊到塞尔维亚大学临床中心神经病学诊所。我们还包括 15 名因不同诊断被转诊给我们的患者,最终他们的最终诊断为 CIDP。被转诊为 CIDP 的患者中有恰好一半未能符合既定的诊断标准(非 CIDP),并在首次住院时被给予替代诊断。在 1 年的随访中,有 4 名患者的诊断进一步修订。确诊的 CIDP 患者通常根据神经传导研究(NCS)做出初始诊断,表现为四肢对称性受累的典型表现,以及脑脊液(CSF)中更高频率的蛋白水平升高和白蛋白细胞分离。CIDP 患者对免疫治疗的反应也更好。我们发现,在 1 年的随访后,最初被转诊到我们诊所的 52%的患者被给予其他诊断。在所有 CIDP 病例中,有 27%在转诊到我们中心之前未被识别。明确和客观指标的利用——明确的 NCS、治疗改善和升高的 CSF 蛋白可能会提供更大的确定性以诊断 CIDP。

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