Dataset related to article "Chronic inflammatory demyelinating polyradiculoneuropathy: can a diagnosis be made in patients not fulfilling electrodiagnostic criteria?"

This record contains data related to article “Chronic inflammatory demyelinating polyradiculoneuropathy: can a diagnosis be made in patients not fulfilling electrodiagnostic criteria?" Abstract <strong>Background and purpose: </strong>The aim was to identify the clinical and diagnostic investigations that may help to support a diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in patients not fulfilling the European Federation of Neurological Societies and Peripheral Nerve Society (EFNS/PNS) electrodiagnostic criteria. <strong>Methods: </strong>The data from patients with a clinical diagnosis of CIDP included in a national database were retrospectively reviewed. <strong>Results: </strong>In all, 535 patients with a diagnosis of CIDP were included. This diagnosis fulfilled the EFNS/PNS criteria in 468 patients (87.2%) (definite in 430, probable in 33, possible in three, while two had chronic immune sensory polyradiculopathy). Sixty-seven patients had a medical history and clinical signs compatible with CIDP but electrodiagnostic studies did not fulfill the EFNS/PNS criteria for CIDP. These patients had similar clinical features and frequency of abnormal supportive criteria for the diagnosis of CIDP compared to patients fulfilling EFNS/PNS criteria. Two or more abnormal supportive criteria were present in 40 (61.2%) patients rising to 54 (80.6%) if a history of a relapsing course as a possible supportive criterion was also included. Increased cerebrospinal fluid proteins and response to immune therapy most frequently helped in supporting the diagnosis of CIDP. Response to therapy was similarly frequent in patients fulfilling or not EFNS/PNS criteria (87.3% vs. 85.9%). <strong>Conclusions: </strong>Patients with a clinical diagnosis of CIDP had similar clinical findings, frequency of abnormal supportive criteria and response to therapy compared to patients fulfilling EFNS/PNS criteria. The presence of abnormal supportive criteria may help in supporting the diagnosis of CIDP in patients with a medical history and clinical signs compatible with this diagnosis but non-diagnostic nerve conduction studies.本数据集关联的论文题为《慢性炎症性脱髓鞘性多发性神经根神经病（chronic inflammatory demyelinating polyradiculoneuropathy, CIDP）：未满足电诊断标准的患者能否确诊？》。摘要 <strong>背景与目的：</strong>本研究旨在明确可辅助诊断未满足欧洲神经病学学会与周围神经学会（European Federation of Neurological Societies and Peripheral Nerve Society, EFNS/PNS）电诊断标准的慢性炎症性脱髓鞘性多发性神经根神经病（CIDP）患者的临床特征与检查指标。<strong>研究方法：</strong>本研究对纳入国家数据库的临床确诊CIDP患者的资料进行回顾性分析。<strong>研究结果：</strong>最终共纳入535例临床确诊CIDP的患者。其中468例（87.2%）满足EFNS/PNS诊断标准（确诊430例，很可能33例，可能3例，另有2例为慢性免疫感觉性神经根神经病）。剩余67例患者的病史与临床体征符合CIDP，但电生理检查未达到EFNS/PNS的CIDP诊断标准。该组患者与满足EFNS/PNS标准的患者相比，临床特征与异常辅助诊断指标的出现频率均无显著差异。40例（61.2%）患者存在2项及以上异常辅助诊断指标，若将复发病程作为潜在辅助诊断指标纳入统计，则该比例可升至54例（80.6%）。脑脊液蛋白升高与免疫治疗应答是最常用于辅助CIDP诊断的指标。满足与未满足EFNS/PNS标准的患者，其治疗应答率无明显差异（87.3% vs 85.9%）。<strong>研究结论：</strong>与满足EFNS/PNS诊断标准的患者相比，临床确诊CIDP的患者在临床特征、异常辅助诊断指标出现频率及治疗应答率方面均无显著差异。对于病史与临床体征符合CIDP但神经传导检查无法确诊的患者，异常辅助诊断指标可辅助其CIDP的诊断。