Néel A, Pagnoux C, Guillevin L, Hamidou M
Service de médecine interne, hôpital Hôtel-Dieu, CHU de Nantes, 1, place Alexis-Ricordeau, 44093 Nantes cedex 01, France.
Rev Med Interne. 2012 Jul;33(7):381-9. doi: 10.1016/j.revmed.2011.11.020. Epub 2012 Jun 7.
Central nervous system (CNS) vasculitides are a heterogeneous group of diseases that can lead to severe disability and death. Given the poor performance of available diagnostic procedures (magnetic resonance imaging, angiography and brain biopsy), the diagnosis of isolated CNS vasculitis is challenging. Differential diagnosis includes secondary CNS vasculitides (infection, cancer, drug exposure or systemic inflammatory disorders) and several non-inflammatory cerebral vasculopathies, such as the reversible cerebral vasoconstriction syndrome. Despite recent advances in neuroradiology and the publication of large retrospective case series, primary angiitis of the CNS remains a heterogeneous and challenging nosological entity. Prospective interdisciplinary studies are necessary to improve our approach to these patients.
中枢神经系统（CNS）血管炎是一组异质性疾病，可导致严重残疾和死亡。鉴于现有诊断程序（磁共振成像、血管造影和脑活检）效果不佳，孤立性中枢神经系统血管炎的诊断具有挑战性。鉴别诊断包括继发性中枢神经系统血管炎（感染、癌症、药物暴露或全身性炎症性疾病）以及几种非炎症性脑血管病，如可逆性脑血管收缩综合征。尽管神经放射学最近取得了进展，并且发表了大量回顾性病例系列，但中枢神经系统原发性血管炎仍然是一个异质性且具有挑战性的疾病实体。需要进行前瞻性跨学科研究，以改进我们对这些患者的治疗方法。
