疑免疫球蛋白G4-相關肥厚硬腦膜炎引起雙側視力喪失

Possible Immunoglobulin G4-Related Hypertrophic Pachy-meningitis Leading To Bilateral Visual Loss

免疫球蛋白G4-相關疾病是一纖維發炎性異常，影響許多器官，最常影響的部位包含有胰臟、肺、甲狀腺、淋巴結、膽管、後腹膜、淚腺、及唾液腺。 肥厚硬腦膜炎為其非常罕見的表現。我們提出一老年男性逐漸無痛性雙眼視力喪失，眼科檢查時右眼視力僅為見手動，左眼已無光覺。眼底檢查發現視神經盤水腫合併視網膜前出血。腦部電腦斷層顯示水腦，脊椎穿刺確定腦壓升高，腦部核磁共振發現左側大腦半球及大腦鐮硬腦膜增厚，血清免疫球蛋白升高，但是硬腦膜切片並無免疫球蛋白G4-相關疾病的病理表現。由於懷疑是免疫球蛋白G4-相關肥厚硬腦膜炎，他開始口服低劑量類固醇治療，可惜治療四個月後追蹤視力及硬腦膜增厚並無改善。

 

Immunoglobulin G4-related disease (IgG4-RD) is a fibro-inflammatory disor-der affecting numerous organs. The most common sites of involvement are pancreas, lungs, thyroid gland, lymph nodes, bile duct, retroperitoneum, and lacrimal and sal-ivary glands. Hypertrophic pachymeningitis is a very rare manifestation of IgG4-RD. He we describe an old man who suffered from painless, progressive bilateral visual loss. On ocular examination, his vision was hand motion in the right eye and no light perception in the left eye. Fundus examination showed severe papilledema with pre-retinal hemorrhage. Brain computed tomography revealed hydrocephalus. Lum-bar puncture confirmed high intra-cranial pressure. Brain magnetic resonance imag-ing uncovered dural thickening at left hemisphere and tentorium. His serum IgG4 level was elevated. However, dural biopsy did not present the hallmarks of IgG4-RD features. As there was suspicion of IgG4-related hypertrophic pachymeningitis, he received oral low-dose steroid treatment. Unfortunately, his vision and thickened dura had not improved after 4 months of steroid treatment.