目的 探讨原发性中枢神经系统血管炎(PACNS)的临床、影像及病理改变特点.方法 收集海军总医院2008-2011年间经病理检查确诊的PACNS患者5例(其中经立体定向脑活检4例,开颅活检l例),通过对患者的所有临床资料进行回顾性分析,总结PACNS的临床、影像、病理改变特点.结果 临床主要表现为头痛、癫痫、视物模糊、言语不利以及局灶的神经功能缺损.影像学表现:幕上4例,幕上下均累及1例；所有脑叶均可累及；单侧发病3例,双侧发病2例.病灶边界模糊,灰白质均可累及,增强扫描后均有不同程度的脑回样或斑片样强化.病理特点为皮质及白质内可见到小血管周围大量的淋巴细胞浸润,多以T淋巴细胞浸润；可见到坏死及吞噬细胞以及反应性星形细胞增生；小血管增生明显,3例患者可见到血管壁的纤维素样坏死.患者经糖皮质激素十免疫抑制剂治疗,疗效满意.结论 PACNS临床表现以局灶的神经功能缺损为主要表现,病变部位为幕上多发,以单侧发病多见.诊断需结合临床特点、实验室、影像学和病理学检查.%Objective To study the clinical, imaging and pathological features of primary angiitis of the central nervous system (PACNS) . Methods Five cases with PACNS were collected in Navy General Hospital from 2008 to 2011, stereotactic brain biopsy was taken in 4 cases and craniotomy in 1 case. The clinical, imaging and pathological characteristics of the 5 PACNS cases were retrospectively analyzed. Results The cases presented with headache, seizures, blurred vision, speech disorder and focal neurological deficits. Brain images showed supratentorial lesions in 4 cases, and both supra- and infratentorial lesions in 1 case. All the cerebral lobes were involved. The lesions were unilateral in 3 cases, and bilateral in 2 cases. The lesions had blurred boundaries, involved gray matter, with various degrees of gyrus-like and patchy enhancement. Pathological features were that a large number of lymphocyte infiltration around small vessels in cortical and white matter, with T lymphocytes predominance. There were necrosis, phagocytes, proliferated astrocytes and pronounced hyperplasia small vessels. Fibrinoid necrosis was seen in vessel walls in three cases. Treatments with glucocorticoid and immunosuppressive agents were satisfied. Conclusions The main clinical manifestation of PACNS was focal neurological deficit. The lesions were mainly multiple and unilateral, located in supratentorium. Diagnosis need to be combined with clinical manifestations, laboratory, imaging and pathological examinations.
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