Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA.
J Neurol Sci. 2012 Jul 15;318(1-2):31-5. doi: 10.1016/j.jns.2012.04.010. Epub 2012 Apr 28.
Orbital apex and skull base masses often present with neuro-ophthalmic signs and symptoms. Though the localization of these syndromes and visualization of the responsible lesion on imaging is typically straightforward, definitive diagnosis usually relies on biopsy. Immunohistochemistry is important for categorization and treatment planning. IgG4-related disease is emerging as a pathologically defined inflammatory process that can occur in multiple organ systems. We present two patients with extensive inflammatory mass lesions of the central nervous system with immunohistochemistry positive for IgG4 and negative for ALK-1 as examples of meningeal based IgG4-related inflammatory pseudotumors. In both patients, there was treatment response to mycophenolate mofetil.
眼眶尖和颅底肿块常表现为神经眼科体征和症状。尽管这些综合征的定位和影像学上责任病变的可视化通常很直接，但明确诊断通常依赖于活检。免疫组织化学对于分类和治疗计划很重要。IgG4 相关疾病是一种在多个器官系统中发生的具有明确病理学定义的炎症过程。我们提出了两例中枢神经系统广泛炎症性肿块病变的患者，其免疫组织化学 IgG4 阳性，ALK-1 阴性，作为脑膜 IgG4 相关炎症性假瘤的例子。在这两例患者中，霉酚酸酯治疗均有反应。
