Chronic inflammatory demyelinating polyneuropathy in end-stage renal disease patients: a case series study.

IF 2.7 4区 医学 Q2 CLINICAL NEUROLOGY
Yize Li, Yakun Wu, Zhenyu Li, Jieyu Li, Ping Li, Zhiying Xie, Yiming Zheng, Jianwen Deng, He Lv, Zhaoxia Wang, Yun Yuan, Lingchao Meng
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Abstract

Objectives: The occurrence of chronic inflammatory demyelinating polyneuropathy (CIDP) in patients with end-stage renal disease (ESRD) has been rarely studied. This study was intended to explore the clinical, electrophysiological and neuropathological features of six patients with CIDP and ESRD.

Methods: The medical records collected in Peking University First Hospital between 2021 and 2024 were reviewed before six patients were identified who had developed CIDP with ESRD. The data on their clinical features, electrophysiological tests, nerve ultrasound, laboratory examinations and sural biopsies was retrieved.

Results: All the six patients were males ages 35 to 63. Before and after dialysis, all these patients presented with varying degrees of limb sensory impairment and weakness, and reduced or absent tendon reflexes. Albuminocytologic dissociation of cerebrospinal fluid was observed in all of them. Nerve conduction studies identified the demyelinating features while nerve ultrasound showed nerve enlargement of upper limbs. Sural nerve biopsies showed mild to moderate decreases of myelinated nerve fiber density, with differences between different nerve fascicles. Thin myelinated fibers were seen, without onion bulb-like structure. Varying degrees of axonal degeneration with regeneration were identified in some of these patients. Immunotherapy was effective in all these patients.

Conclusions: The diagnosis of CIDP should be considered if an ESRD patient develops progressive demyelinating neuropathy. Immune therapy has shown clinical efficacy despite the underlying mechanisms that remain poorly understood.

终末期肾病患者慢性炎性脱髓鞘性多神经病变:病例系列研究。
目的:慢性炎症性脱髓鞘性多神经病变(CIDP)在终末期肾病(ESRD)患者中的发生很少被研究。本研究旨在探讨6例CIDP合并ESRD患者的临床、电生理和神经病理特征。方法:回顾性分析北京大学第一医院于2021 - 2024年间收集的6例CIDP合并ESRD患者的病历。检索他们的临床特征、电生理检查、神经超声、实验室检查和腓肠活检的资料。结果:6例患者均为男性,年龄35 ~ 63岁。透析前后患者均出现不同程度的肢体感觉障碍和无力,肌腱反射减少或缺失。所有患者脑脊液均有白蛋白细胞分离。神经传导检查发现脱髓鞘特征,神经超声显示上肢神经肿大。腓肠神经活检显示有髓神经纤维密度轻度至中度降低,不同神经束间存在差异。可见薄髓鞘纤维,无洋葱鳞茎样结构。在这些患者中发现了不同程度的轴突变性和再生。免疫治疗对所有患者均有效。结论:如果ESRD患者发生进展性脱髓鞘神经病变,应考虑CIDP的诊断。免疫疗法已显示出临床疗效,尽管其潜在机制尚不清楚。
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来源期刊
Neurological Sciences
Neurological Sciences 医学-临床神经学
CiteScore
6.10
自引率
3.00%
发文量
743
审稿时长
4 months
期刊介绍: Neurological Sciences is intended to provide a medium for the communication of results and ideas in the field of neuroscience. The journal welcomes contributions in both the basic and clinical aspects of the neurosciences. The official language of the journal is English. Reports are published in the form of original articles, short communications, editorials, reviews and letters to the editor. Original articles present the results of experimental or clinical studies in the neurosciences, while short communications are succinct reports permitting the rapid publication of novel results. Original contributions may be submitted for the special sections History of Neurology, Health Care and Neurological Digressions - a forum for cultural topics related to the neurosciences. The journal also publishes correspondence book reviews, meeting reports and announcements.
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