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RELEVANCE OF DIAGNOSTIC INVESTIGATIONS IN CHRONIC INFLAMMATORY DEMYELINATING POLIRADICULONEUROPATHY: DATA FROM THE ITALIAN CIDP DATABASE.
Journal of the Peripheral Nervous System ( IF 3.9 ) Pub Date : 2020-05-13 , DOI: 10.1111/jns.12378
Giuseppe Liberatore 1 , Fiore Manganelli 2 , Dario Cocito 3 , Raffaella Fazio 4 , Chiara Briani 5 , Massimiliano Filosto 6 , Luana Benedetti 7, 8 , Giovanni Antonini 9 , Giuseppe Cosentino 10 , Stefano Jann 11 , Anna Mazzeo 12 , Andrea Cortese 13 , Girolama Alessandra Marfia 14 , Angelo Maurizio Clerici 15 , Gabriele Siciliano 16 , Marinella Carpo 17 , Mario Sabatelli 18, 19 , Giuseppe Lauria 20, 21 , Tiziana Rosso 22 , Eduardo Nobile Orazio 1, 23 ,
Affiliation  

The objective of our work was to report the clinical features and the relevance of diagnostic investigations in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We retrospectively reviewed data from patients with a clinical diagnosis of CIDP included in a national database. Among the 500 included patients with a clinical diagnosis of CIDP, 437 patients (87%) fulfilled the European Federation of Neurological Societies and Peripheral Nerve Society criteria for CIDP (definite in 407, probable in 26, possible in four). In 352 patients (86%) motor nerve conduction abnormalities consistent with demyelination were sufficient for the diagnosis of definite CIDP. In 55 patients, this diagnosis required the addition of one or two (from probable or from possible CIDP, respectively) supportive tests, while in 20 cases they improved the diagnosis from possible to probable CIDP, seven patients did not change diagnosis. Considering these 85 patients, cerebrospinal fluid studies were performed in 79 cases (93%) upgrading the certainty of diagnosis in 59% of examined patients. Sensory nerve conduction studies (NCS) were performed in 85% of patients with an improvement of diagnosis in 32% of cases. Nerve biopsy and ultrasound and magnetic resonance imaging (US/MRI) exams resulted positive in about 40% of examined patients, but they were performed in few patients (7 patients and 16 patients, respectively). A response to the therapy was present in 84% of treated patients (n = 77), contributing to support the diagnosis in 40 patients in whom the other supportive criteria were not sufficient. In most patients with CIDP the diagnosis is possible solely with motor NCS while other investigations may help improving the diagnosis in a minority of patients.

中文翻译:


慢性炎症性脱髓鞘性多发性神经根神经病诊断调查的相关性:来自意大利 CIDP 数据库的数据。



我们工作的目的是报告慢性炎症性脱髓鞘性多发性神经根神经病 (CIDP) 患者的临床特征和诊断研究的相关性。我们回顾性审查了国家数据库中包含的 CIDP 临床诊断患者的数据。在 500 名临床诊断为 CIDP 的患者中,437 名患者 (87%) 符合欧洲神经学会和周围神经学会联合会的 CIDP 标准(确定为 407 名,可能为 26 名,可能为 4 名)。在 352 名患者 (86%) 中,与脱髓鞘一致的运动神经传导异常足以诊断明确的 CIDP。在 55 名患者中,这一诊断需要增加一到两项(分别来自可能或可能 CIDP)支持性测试,而在 20 例患者中,他们将诊断从可能改善为可能 CIDP,7 名患者没有改变诊断。考虑到这 85 名患者,对 79 例 (93%) 进行了脑脊液检查,提高了 59% 受检查患者的诊断确定性。 85% 的患者进行了感觉神经传导研究 (NCS),其中 32% 的病例诊断得到改善。约 40% 的受检查患者的神经活检以及超声和磁共振成像 (US/MRI) 检查结果呈阳性,但在少数患者中进行了这些检查(分别为 7 名患者和 16 名患者)。 84% 的治疗患者 (n = 77) 对治疗有反应,这有助于支持 40 名其他支持标准不充分的患者的诊断。对于大多数 CIDP 患者,仅通过运动 NCS 即可进行诊断,而其他检查可能有助于改善少数患者的诊断。
更新日期:2020-05-13
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