Tomio Ryosuke, Ohira Takayuki, Wenlin Du, Yoshida Kazunari
Department of Neurosurgery, Keio University School of Medicine, Shinjuku, Japan.
BMJ Case Rep. 2013 Jan 11;2013:bcr2012007320. doi: 10.1136/bcr-2012-007320.
We report the first documented case of IgG4-related inflammatory pseudotumours (IPTs) along the bilateral oculomotor nerves. A man in his 60s complained of decreased vision. He exhibited bilateral optic nerve atrophy without any extraocular movement deficits. MRI revealed enhanced masses that reached from the bilateral cavernous sinus to within the bilateral orbits. The tumours extended along the lines of the bilateral oculomotor nerves. The patient's serum level of IgG4 was high, 147 mg/dl. A biopsy specimen showed inflammatory cell-rich lesions against a collagenous stroma. Immunostaining revealed infiltration of CD138-positive plasma cells, which were mainly IgG and IgG4 positive. The IgG4/IgG ratio was greater than 0.4. These factors led us to a diagnosis of IgG4-related IPTs. Oral administration of prednisolone (30 mg/day) was started 3 months after the operation and continued for 6 months with gradual tapering. The tumour was significantly reduced by prednisolone.
我们报告首例有文献记载的双侧动眼神经IgG4相关性炎性假瘤（IPT）病例。一名60多岁男性主诉视力下降。他表现为双侧视神经萎缩，无任何眼球运动障碍。磁共振成像（MRI）显示强化肿块，从双侧海绵窦延伸至双侧眼眶内。肿瘤沿双侧动眼神经走行扩展。患者血清IgG4水平较高，为147mg/dl。活检标本显示富含炎症细胞的病变，伴有胶原性基质。免疫染色显示CD138阳性浆细胞浸润，主要为IgG和IgG4阳性。IgG4/IgG比值大于0.4。这些因素使我们诊断为IgG4相关性IPT。术后3个月开始口服泼尼松龙（30mg/天），持续6个月并逐渐减量。泼尼松龙使肿瘤明显缩小。
