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Primary angiitis of the central nervous system: 2 atypical cases

机译:中枢神经系统原发性血管炎:2例非典型病例

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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 chro nic 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.
机译:中枢神经系统(CNS)中血管炎过程的存在表征了不同的疾病组:从特发性脑膜炎到淋巴增生性疾病。在没有特定的感染,炎性和肿瘤性疾病的情况下,提出了术语“ PACNS”(中枢神经系统原发性血管炎)来表示未知来源的脑膜血管延伸到大脑或脊髓实质的特殊血管炎症。我们报告了2例具有特殊和非典型特征的PACNS:第一例可能与爱泼斯坦巴尔病毒(EBV)相关,第二例仅涉及脊髓,均通过手术治疗。我们还假设EBV慢性感染与PACNS的可能亚型之间存在相关性,从而强调了EBER(EBV编码的RNA)测试在对PACNS可疑的脑活检的常规检查中的重要性。

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