Neuro-surgical considerations for treating IgG4-related disease with rare spinal epidural compression.
Neuro-surgical considerations for treating IgG4-related disease with rare spinal epidural compression.
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DOI：
10.4103/sni.sni_156_18
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发表时间：
2018
影响因子：
--
通讯作者：
Smith ZA
中科院分区：
文献类型：
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作者：
Winkel M;Lawton CD;Sanusi OR;Horbinski CM;Dahdaleh NS;Smith ZA
免疫球蛋白g4相关疾病（IgG4-RD）是一组独特的自身免疫性疾病，几乎影响身体的每个器官系统。虽然中枢神经系统受累是相当罕见的，它可能表现为肥厚性厚性脑膜炎更频繁地影响大脑而不是脊柱。在这项研究中，我们提供了一例脊髓IgG4-RD假肿瘤导致脊髓压迫，并对文献进行了全面的回顾。患者表现为硬膜外肿块，导致L2-L3水平脊髓受压。病理证实为IgG4-RD假瘤。患者接受鞘囊减压术和术后类固醇治疗，症状完全缓解。IgG4-RD在脊柱文献中通常未被充分认识和报道。脊柱的临床表现和非手术与手术治疗相对简单。虽然大多数病例可以通过一个疗程的类固醇治疗，但出现脊髓和/或神经根压迫的患者可能需要手术减压。这些脊柱肿瘤或假肿瘤的鉴别诊断应包括IgG4-RD。早期发现和适当治疗可导致令人满意的结果。
Immunoglobulin G4-related disease (IgG4-RD) is a group of distinct autoimmune disorders affecting nearly every organ system in the body. Although central nervous system involvement is quite rare, it may present as hypertrophic pachymeningitis more frequently affecting the brain than the spine. In this study, we provide a case of spinal IgG4-RD pseudotumor resulting in cord compression, and a comprehensive review of the literature. A patient presented with an extradural mass causing spinal cord compression at the L2-L3 level. Pathologically this proved to be an IgG4-RD pseudotumor. The patient was treated with thecal sac decompression and post-operative steroids that resulted in complete resolution of his symptoms. IgG4-RD is typically under-recognized and under-reported in the spinal literature. The clinical spinal presentation and non-surgical vs. surgical treatment are relatively straightforward. Although most cases can be managed with a course of steroids, surgical decompression may be required in patients presenting with spinal cord and/or nerve root compression. The differential diagnoses for these spinal tumors or pseudotumors should include IgG4-RD. Early detection and appropriate treatment can lead to satisfactory outcomes.