原发性中枢神经系统血管炎的临床及影像学分析

目的探讨原发性中枢神经系统血管炎的临床和影像学表现，以明确诊断要点。方法临床或病理证实的中枢神经系统血管炎8例，其中男性3例，女性5例。年龄从19～73不等，平均年龄40．3岁。全部行MRI检查，其中增强4例；8例行MRA检查，分析所有病例的临床资料和MR片。结果8例PACNS中，8例位于幕上，以额叶及顶叶多见，其中3例为双侧多发；幕下4例位于脑干及脊髓。影像学上，多数病灶边界模糊，与相应血管分布区一致，累及灰质和自质，但以白质为主，可有水肿，增强后有不同程度增强，呈斑片样或脑回样，甚至呈结节样，类似肿瘤。血管成像可以发现血管炎的征象。结论原发性中枢神经系统血管炎的临床和影像学表现多样，MRI具有一定价值，MRA及DSA对诊断帮助较大，确诊需要活检病理证实。

Clinical Analysis and MRI Features of Primary Angiitis of the Central Nervous System

Objective To study the clinical and imaging feature of primary angiitis of the central nervous system(PACNS),in order to find the key point of diagnosis.Materials and Methods Pathological or clinical proved PACNS 9 cases,3 men and 5 women,ranging from 19～73 years old with average 40.2 years old.All were examined with MRI and 4 by enhanced scan:8 with MRA and 2 with DSA.Clinical information and all films(MRI and DSA)were analyzed.Results In 8 cases of PACNS,1cases were proved by operation or biopsy,and 7 cases were proved by clinical follow-up.8 cases were located supratentorial,most were in frontal lobe,3 cases were multiple focuses in both sides;4 cases were located infratentorial and brain sten and spinal.On imaging,most focuses were with blurry margin,and the regions were accordant with that of the blood vessel.The lesions involved gray matter and white matter,but mostly in white matter,edema could be seen in some cases.Different grade of enhancement could be seen,the enhancement may be patchy or convolutional,even nodulous witch was resemble with tumor.The sign of angiitis could be seen in angiography.Conclusion The clinical and imaging features of PACNS are various,CT and MRI ale valuable,MRA and DSA are helpful in diagnosis,but the diagnosis must given by pathological examination.