Centro per le Malattie Rare e Neuroimmuni, Unità Operativa di Neurologia, Dipartimento di Neuroscienze, Ospedale Bellaria, Bologna, Italy.
Neurol Sci. 2010 Oct;31(5):639-41. doi: 10.1007/s10072-010-0283-8. Epub 2010 May 18.
Isolated bilateral optic neuropathy is an exceedingly rare presentation of perinuclear-antineutrophil cytoplasmic autoantibody (p-ANCA) vasculitis. We report one such case with MRI documentation of a compressive mechanism mediated by pachymeningitis. A 69-year-old woman had a 6-month history of progressive visual failure caused by bilateral optic neuropathy. Cranial MRI showed diffuse contrast enhancement of the dura mater extending to the perioptic sheaths bilaterally with encasement of the optic nerves. Extensive laboratory study showed increased levels of p-ANCA titres and autoimmune markers. Corticosteroid treatment improved both visual acuity and the neuroradiological signs of active pachymeningitis. In conclusion, our case demonstrates that bilateral optic neuropathy may be the presenting symptom of p-ANCA-associated pachymeningitis, whose causative role may be demonstrated by MRI.
孤立性双侧视神经病变是核周抗中性粒细胞胞浆抗体（p-ANCA）血管炎的一种极其罕见的表现。我们报告了一例 p-ANCA 血管炎伴脑脊髓膜炎引起压迫机制的病例，并通过 MRI 进行了记录。一名 69 岁女性因双侧视神经病变导致进行性视力丧失 6 个月。颅脑 MRI 显示硬脑膜弥漫性对比增强，双侧向眶周鞘延伸，视神经受压。广泛的实验室研究显示 p-ANCA 滴度和自身免疫标志物水平升高。皮质类固醇治疗改善了视力和活动性脑脊髓膜炎的神经放射学征象。总之，我们的病例表明，双侧视神经病变可能是 p-ANCA 相关脑脊髓膜炎的首发症状，MRI 可证实其病因。
