Orthopedic and Rheumatologic Institute, Rheumatologic and Immunologic, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA.
Curr Atheroscler Rep. 2013 Aug;15(8):346. doi: 10.1007/s11883-013-0346-4.
Primary angiitis of the central nervous system (PACNS) is one of the most devastating pathologic processes that affect the central nervous system (CNS). It results in exclusive inflammation and destruction of CNS blood vessels. Progressive debilitating unexplained neurological deficit associated with abnormal cerebrospinal fluid (CSF) analysis findings is the typical picture of the disease. CNS biopsy is the gold standard diagnostic test. Immunosuppressive therapy is the core treatment. Reversible cerebral vasoconstriction syndrome (RCVS) is a main mimic of PACNS. RCVS is characterized clinically by recurrent thunderclap headache with or without neurological deficit and normal CSF analysis findings and angiographically by reversible diffuse segmental vasospasm of intracranial vessels. A stepwise diagnostic approach should be followed to differentiate PACNS from RCVS and exclude the other clinical, radiographic, and angiographic mimics.
原发性中枢神经系统血管炎（PACNS）是影响中枢神经系统（CNS）的最具破坏性的病理过程之一。它导致中枢神经系统血管的炎症和破坏。与异常脑脊液（CSF）分析结果相关的进行性、使人虚弱且无法解释的神经功能缺损是该疾病的典型表现。中枢神经系统活检是金标准诊断测试。免疫抑制疗法是核心治疗方法。可逆性大脑血管收缩综合征（RCVS）是 PACNS 的主要模拟疾病。RCVS 的临床特征为反复发作的霹雳样头痛，伴有或不伴有神经功能缺损和正常的 CSF 分析结果，血管造影表现为颅内血管的可逆性弥漫性节段性血管痉挛。应采用逐步诊断方法来区分 PACNS 和 RCVS，并排除其他临床、影像学和血管造影模拟疾病。
