Ferro J M
Hospital Santa Maria, Faculdade de Medicina de Lisboa, Portugal.
J Neurol. 1998 Dec;245(12):766-76. doi: 10.1007/s004150050285.
Central nervous system (CNS) involvement and management in primary vasculitis (giant cell arteritis, primary angiitis of the CNS, Takayasu's disease, periarteritis nodosa, Kawasaki disease, Churg-Strauss syndrome, Wegener's granulomatosis) and vasculitis secondary to collagen vascular diseases, Beçhet's disease and other systemic conditions (excluding those secondary to infections) and the use of illicit drugs are reviewed. Vasculitis is an infrequent cause of stroke even in the young age groups, and routine screening of stroke patients for vasculitis is not cost-effective. CNS vasculitis may present with isolated CNS symptoms and signs (headaches, meningeal signs, encephalopathy, psychiatric syndromes, dementia, cranial nerve palsies, seizures, strokes), neuropathy or muscle damage, multiorgan involvement or non-specific systemic symptoms or a combination of the above. Magnetic resonance is the most sensitive ancillary procedure to detect CNS damage. Many of the angiographic features found in vasculitis are non-specific. Autoantibodies and tissue biopsy are also useful to the diagnosis. There are few controlled studies on the treatment of vasculitis. Usually a combination of steroids and cytotoxic drugs is used, but there is considerable variation between centres on current therapeutic regimens.
本文综述了原发性血管炎（巨细胞动脉炎、中枢神经系统原发性血管炎、高安氏病、结节性多动脉炎、川崎病、变应性肉芽肿性血管炎、韦格纳肉芽肿）、胶原血管病继发的血管炎、白塞病及其他全身性疾病（不包括感染继发的疾病）以及使用非法药物所致血管炎时中枢神经系统（CNS）的受累情况及处理。血管炎即使在年轻人群中也是导致中风的罕见原因，对中风患者常规筛查血管炎并不具有成本效益。中枢神经系统血管炎可能表现为孤立的中枢神经系统症状和体征（头痛、脑膜刺激征、脑病、精神综合征、痴呆、颅神经麻痹、癫痫发作、中风）、神经病变或肌肉损伤、多器官受累或非特异性全身症状，或上述情况的组合。磁共振成像（MRI）是检测中枢神经系统损伤最敏感的辅助检查方法。血管炎中发现的许多血管造影特征是非特异性的。自身抗体和组织活检对诊断也有帮助。关于血管炎治疗的对照研究很少。通常使用类固醇和细胞毒性药物联合治疗，但目前各中心的治疗方案差异很大。
