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一例酷似吉兰-巴雷综合征的非系统性血管炎性神经病罕见病例报告

A Rare Presentation of Non-systemic Vasculitic Neuropathy Mimicking Guillain-Barré Syndrome: A Case Report.

作者信息

Munagama Chathuri L, Wathurapatha Wasundara S, Rajendiran Varithamby T, Silva Shehan

机构信息

University Medical Unit, Colombo South Teaching Hospital, Colombo, LKA.

Neurology, Colombo South Teaching Hospital, Colombo, LKA.

出版信息

Cureus. 2024 Feb 26;16(2):e54945. doi: 10.7759/cureus.54945. eCollection 2024 Feb.

DOI:10.7759/cureus.54945
PMID:38544597
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10968341/
Abstract

Vasculitic neuropathy typically presents as a painful, asymmetrical sensory-motor polyneuropathy, more commonly demonstrating a mononeuritis multiplex. We present the case of a 63-year-old woman who experienced acute-onset flaccid weakness in all four limbs following an episode of diarrhea. Guillain-Barré syndrome (GBS) was considered, which supported acute motor axonal neuropathy (AMAN) in the nerve conduction study (NCS). On the second day of treatment with intravenous immunoglobulin (IVIG), a vasculitic-type rash appeared along with limb pain. Furthermore, the asymmetrical sensory and motor weakness did not respond well to the treatment. A positive skin biopsy, however, with a negative nerve biopsy combined with repeat NCSs demonstrating mononeuritis multiplex, confirmed the diagnosis of non-systemic vasculitic neuropathy (NSVN) based upon Brighton Case Collaboration type 3. This presentation underlines the significance of considering vasculitic neuropathy as a potential diagnosis and highlights the importance of an accurate diagnosis, as this condition can be effectively treated.

摘要

血管炎性神经病通常表现为疼痛性、不对称性感觉运动性多发性神经病,更常见的是表现为多发性单神经炎。我们报告一例63岁女性病例,该患者在一次腹泻发作后出现四肢急性弛缓性无力。考虑为吉兰-巴雷综合征(GBS),神经传导研究(NCS)支持急性运动轴索性神经病(AMAN)。在静脉注射免疫球蛋白(IVIG)治疗的第二天,出现了血管炎型皮疹并伴有肢体疼痛。此外,不对称的感觉和运动无力对治疗反应不佳。然而,皮肤活检阳性,神经活检阴性,结合重复NCS显示多发性单神经炎,根据布莱顿病例协作组3型确诊为非系统性血管炎性神经病(NSVN)。本病例强调了将血管炎性神经病作为潜在诊断的重要性,并突出了准确诊断的重要性,因为这种疾病可以得到有效治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b1a/10968341/9fb3a48e2a28/cureus-0016-00000054945-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b1a/10968341/ea9ff72fa483/cureus-0016-00000054945-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b1a/10968341/12d81cfca67f/cureus-0016-00000054945-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b1a/10968341/c4a4d1edc739/cureus-0016-00000054945-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b1a/10968341/9fb3a48e2a28/cureus-0016-00000054945-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b1a/10968341/ea9ff72fa483/cureus-0016-00000054945-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b1a/10968341/12d81cfca67f/cureus-0016-00000054945-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b1a/10968341/c4a4d1edc739/cureus-0016-00000054945-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b1a/10968341/9fb3a48e2a28/cureus-0016-00000054945-i04.jpg

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本文引用的文献

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Efficacy and safety of Rituximab in vasculitic neuropathy: a systematic review of the literature.利妥昔单抗治疗血管炎性神经病的疗效与安全性:文献系统综述
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Vasculitic peripheral neuropathy: Case definition and guidelines for collection, analysis, and presentation of immunisation safety data.血管炎性周围神经病:免疫接种安全性数据的病例定义及收集、分析和报告指南
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Non-Systemic Vasculitic Neuropathy: An Enigmatic Clinical Entity.非系统性血管炎性神经病:一种神秘的临床病症。
Am J Case Rep. 2015 Jul 13;16:449-53. doi: 10.12659/AJCR.894601.
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Diagnosis and therapeutic options for peripheral vasculitic neuropathy.外周血管炎性神经病的诊断与治疗选择
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Peripheral Nerve Society Guideline on the classification, diagnosis, investigation, and immunosuppressive therapy of non-systemic vasculitic neuropathy: executive summary.周围神经学会关于非系统性血管炎性神经病的分类、诊断、检查和免疫抑制治疗指南:执行摘要。
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Skin biopsy as an additional diagnostic tool in non-systemic vasculitic neuropathy.皮肤活组织检查在非系统性血管炎性神经病中的辅助诊断作用。
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