Department of Neurology, Japanese Red Cross Medical Center, Japan.
J Neuroimmunol. 2012 Sep 15;250(1-2):115-7. doi: 10.1016/j.jneuroim.2012.05.006. Epub 2012 May 29.
This report describes a rare case of hypertrophic pachymeningitis secondary to antiphospholipid syndrome in a 37-year-old woman. The patient had a history of antiphospholipid syndrome and developed hypertrophic pachymeningitis subsequently. Pathological examination of the dura mater showed not only fibrous thickening, the typical finding of hypertrophic pachymeningitis, but also thrombosis in the dural microvessels and T cell infiltration without B cell or plasma cell infiltration, suggesting cell-mediated immunity. The dural thickening spontaneously improved and did not deteriorate during corticosteroid therapy. The histological findings and spontaneous remission observed in this case might be characteristic of secondary hypertrophic pachymeningitis with antiphospholipid syndrome. Further investigations are necessary to elucidate the pathophysiology of this novel type of hypertrophic pachymeningitis.
本报告描述了一例 37 岁女性抗磷脂综合征继发肥厚性硬脑膜炎的罕见病例。该患者有抗磷脂综合征病史,随后发生肥厚性硬脑膜炎。硬脑膜的病理检查不仅显示出纤维性增厚,这是肥厚性硬脑膜炎的典型表现,还显示出硬脑膜微血管内血栓形成和 T 细胞浸润,而没有 B 细胞或浆细胞浸润,提示细胞介导的免疫。硬脑膜增厚在皮质类固醇治疗期间自发改善,并未恶化。该病例的组织学发现和自发缓解可能是抗磷脂综合征继发肥厚性硬脑膜炎的特征。进一步的研究对于阐明这种新型肥厚性硬脑膜炎的病理生理学是必要的。