IgG4 相关脊髓肥厚性硬脑膜炎伴神经功能缺损：2 例报告。
IgG4-Related Spinal Hypertrophic Pachymeningitis With Neurological Deficit: A Report of 2 Cases.
机构信息
Department of Orthopedic Surgery, Maharat Nakhon Ratchasima Hospital, Nakhon Ratchasima, Thailand.
Department of Hematology, Maharat Nakhon Ratchasima Hospital, Nakhon Ratchasima, Thailand.
出版信息
JBJS Case Connect. 2023 Jun 1;13(2). doi: e23.00024. eCollection 2023 Apr 1.
CASE
Immunoglobulin G4 (IgG4)-related spinal hypertrophic pachymeningitis (IgG4-RHP) is a rare disease characterized by diffuse inflammatory fibrosis of the spinal dura mater that can cause myelopathy and neurological deficits. Here, we report 2 cases in which both patients presented with myelopathy and paraplegia. They underwent decompressive laminectomy, and the diagnoses were confirmed through histopathologic examination. Both patients received prednisolone postoperatively, which was subsequently switched to methotrexate for maintenance.
CONCLUSION
Two cases of IgG4-RHP were successfully treated with decompressive laminectomy and combined therapy with steroids and other immunosuppressive agents.
病例
免疫球蛋白 G4（IgG4）相关的脊柱肥厚性硬脑膜炎（IgG4-RHP）是一种罕见疾病，其特征为脊柱硬脑膜弥漫性炎症性纤维化，可导致脊髓病和神经功能缺损。在此，我们报告 2 例均表现为脊髓病和截瘫的患者。他们接受了减压椎板切除术，通过组织病理学检查确诊。术后，这 2 例患者均接受泼尼松龙治疗，随后改用甲氨蝶呤维持治疗。
结论
2 例 IgG4-RHP 患者通过减压椎板切除术和类固醇及其他免疫抑制剂联合治疗获得成功。
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