Department of Pathology, Università Milano Bicocca, San Gerardo Hospital, Monza, Italy.
Folia Neuropathol. 2012;50(3):293-9. doi: 10.5114/fn.2012.30530.
The presence of an angiitis process in the central nervous system (CNS) characterizes different groups of conditions: from idiopathic pachymeningitis to lymphoproliferative disorders. In absence of specific infections, inflammatory and neoplastic diseases, the term "PACNS" (Primary Angiitis of the CNS) was proposed to indicate a peculiar vascular inflammation of unknown origin of meningeal vessels extending to the brain or spinal cord parenchyma. We report two cases of PACNS with peculiar and atypical features: the first one with a possible Epstein Barr Virus (EBV) relationship, the second one with spinal cord involvement only, treated surgically. We also hypothesize a correlation between EBV chronic infection and possible subtypes of PACNS stressing the importance of EBER (EBV-encoded RNA) test in the routine examination of brain biopsies suspicious for PACNS.
中枢神经系统（CNS）血管炎的存在可将不同疾病区分开来：从特发性肥厚性硬脑膜炎到淋巴增生性疾病。在无特殊感染、炎症和肿瘤性疾病的情况下，提出“PACNS”（中枢神经系统原发性血管炎）一词，用于表示脑膜血管的一种来源不明的特殊血管炎症，可延伸至脑或脊髓实质。我们报告了两例具有特殊和非典型特征的 PACNS 病例：一例可能与 Epstein Barr 病毒（EBV）有关，另一例仅累及脊髓，行手术治疗。我们还假设 EBV 慢性感染与可能的 PACNS 亚型之间存在相关性，强调在对疑似 PACNS 的脑活检进行常规检查时进行 EBER（EBV 编码 RNA）检测的重要性。
