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表现为周围神经病变的免疫球蛋白G4相关性疾病:一种由罕见自身免疫性疾病引起的罕见临床症状。

Immunoglobulin G4-related disease manifesting as peripheral neuropathy: A rare clinical symptom due to rare autoimmune disease.

作者信息

Kobayashi Tamaki, Maki Yosinori, Ikeda Hiroyuki, Koyanagi Masaomi, Oda Masashi, Saiki Masaaki

机构信息

Department of Spinal Neurosurgery, Kyoto-Katsura Hospital, Kyoto, Japan.

Department of Neurosurgery, Hikone Chuo Hospital, Hikone, Japan.

出版信息

Surg Neurol Int. 2024 Jun 14;15:197. doi: 10.25259/SNI_157_2024. eCollection 2024.

Abstract

BACKGROUND

Nervous system involvement in immunoglobulin G4-related disease (IgG4-RD) has been rarely reported.

CASE DESCRIPTION

We describe an unusual case of IgG4-RD manifested as paresthesia in the right lower extremity. A 51-year-old male presented with paresthesia in the right S1-S3 regions. A neurological examination revealed peripheral neuropathy. Blood examination results were normal, barring slightly elevated IgG levels. Initial magnetic resonance imaging of the swollen right S1 and S2 nerve roots revealed lymphoma, schwannoma, and sarcoidosis. However, following the biopsy, the pathological findings were not typical of these diseases. Abdominal computed tomography revealed perirenal lesions, and IgG4-RD was suspected. The patient had a serum IgG4 level of 724 mg/dL. Additional pathological evaluations of the swollen S1 nerve revealed findings that corresponded to the diagnostic criteria for IgG4-RD. Oral steroid therapy was initiated, which improved paresthesia, and the swollen S1 nerve root gradually shrank.

CONCLUSION

This report highlights a rare case o IgG4-RD involving nerve roots that neurosurgeons should consider.

摘要

背景

免疫球蛋白G4相关性疾病(IgG4-RD)累及神经系统的报道罕见。

病例描述

我们描述了1例表现为右下肢感觉异常的罕见IgG4-RD病例。一名51岁男性,表现为右侧S1-S3区域感觉异常。神经系统检查显示为周围神经病变。血液检查结果正常,仅IgG水平略有升高。最初对肿胀的右侧S1和S2神经根进行的磁共振成像显示为淋巴瘤、神经鞘瘤和结节病。然而,活检后,病理结果并不符合这些疾病的典型表现。腹部计算机断层扫描显示肾周病变,怀疑为IgG4-RD。患者血清IgG4水平为724mg/dL。对肿胀的S1神经进行的进一步病理评估显示符合IgG4-RD的诊断标准。开始口服类固醇治疗后,感觉异常得到改善,肿胀的S1神经根逐渐缩小。

结论

本报告强调了1例罕见的累及神经根的IgG4-RD病例,神经外科医生应予以考虑。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dbf2/11225528/fdae0cf3eccb/SNI-15-197-g001.jpg

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