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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.

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/ea9ff72fa483/cureus-0016-00000054945-i01.jpg

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