Pathological Findings in Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Single-Center Experience
Pathological Findings in Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Single-Center Experience
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DOI：
10.3390/brainsci10060383
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发表时间：
2020-06-01
期刊：
影响因子：
3.3
通讯作者：
Sabatelli, Mario
中科院分区：
文献类型：
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作者：
Luigetti, Marco;Romano, Angela;Sabatelli, Mario
目的：节段性脱髓鞘是慢性炎性脱髓鞘性多发性神经根神经病（CIDP）的病理特征，但也常伴有其他原发性病变，形成一系列不同的病理形态。在这篇文章中，我们回顾了一个大系列的腓肠神经活检的病理结果，从我们的队列CIDP患者。患者和方法：从1982年至2020年2月在意大利罗马的“卡托利卡德尔圣心大学”神经病学研究所转诊的患者中回顾性选择接受神经活检的CIDP患者。根据标准方案进行腓肠神经活检。结果：130例CIDP患者中有43例进行了腓肠神经活检。脱髓鞘异常和轴突丢失分别在67.4%和83.7%的活检中发现。相反，洋葱球和炎性浸润是罕见的（分别为18.6%和4.7%）。在3例病例中，我们观察到正常的病理结果。结论：CIDP的特异性病理发现不能建立，但我们证实了神经活检在这种情况下的效用，以确认诊断（也在非典型表型），并阐明致病机制。
Objective: Segmental demyelination is the pathological hallmark of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), but other elementary lesions are frequently observed, configuring a series of different pathological pictures. In this article, we review the pathological findings of a large series of sural nerve biopsies from our cohort of CIDP patients. Patients and Methods: Patients with CIDP who underwent nerve biopsy were retrospectively selected from those referred to the Institute of Neurology of the "Universita Cattolica del Sacro Cuore" in Rome, Italy, from 1982 to February 2020. Sural nerve biopsy was performed according to standard protocols. Results: Sural nerve biopsy was performed in 43/130 CIDP patients. Demyelinating abnormalities and axonal loss were found in 67.4% and 83.7% of biopsies, respectively. Conversely, onion bulbs and inflammatory infiltrates were rare (18.6% and 4.7%, respectively). In three cases, we observed normal pathological findings. Conclusions: A pathognomonic pathological finding of CIDP cannot be established, but we confirm the utility of nerve biopsy in this setting to confirm the diagnosis (also in atypical phenotypes) and to elucidate pathogenic mechanisms.