原发性中枢神经系统血管炎的影像表现兼与可逆性后部白质脑病比较

The Imaging Manifestations of Primary Angiitis of Central Nervous System and Comparison with Reversible Posterior Leukoencephalopathy Syndrome

目的分析原发性中枢神经系统血管炎与可逆性后部白质脑病的临床与影像表现,提高对其认识水平。方法搜集经临床随访证实的原发性中枢神经系统血管炎2例和可逆性后部白质脑病4例,对比分析其临床及影像表现。结果原发性中枢神经系统血管炎病灶分布以基底节区、脑干及胼胝体等深部脑白质为主,可不对称性累及额、颞、顶、枕叶;可逆性后部白质脑病早期病灶对称性分布于额、顶、枕叶,病程进展可累及基底节等深部脑白质。CT平扫表现为低密度灶,MRI平扫信号均表现为T1WI呈略低信号,T2WI、FLAIR及DWI均呈略高信号,ADC图呈等或略高信号;原发性中枢神经系统血管炎增强扫描呈斑片状强化,可伴血管多发节段性狭窄及小动脉瘤。结论原发性中枢神经系统血管炎及可逆性后部白质脑病影像表现有一定的特征,结合临床病史能准确诊断。

Objective To analyze the clinic and imaging manifestations of primary angiitis of the central nervous system （PACNS） and reversible posterior leukoencephalopathy syndrome （RPLS） in order to improve the understanding of these diseases. Methods Two patients with clinically-confirmed PACNS and four patients with clinically-proved RPLS were enrolled in this study. The clinical and imaging manifestations of both diseases were retrospectively analyzed, and the results were compared between the two diseases. Results The PACNS lesions mainly distributed in the deep brain white matter, such as basal ganglia, brain stem and corpus callosum, et al. The lobus of fromatis, temporalis, parietalis, and occipita could be asymmetrically involved. The early lesions of RPLS usually symmetrically distributed in the lobus of fromatis, parietalis, and occipita. With the disease progressing, the deep brain white matter such as basal ganglia could be involved. They showed low density on CT scan, and displayed slightly hypointensity on T1WI, shghtly hyperintensity on T2WI, FLAIR, DWI and ADC of MR scan. The lesions of PACNS showed patchy enhancement, and the CTA showed multiple segmental vascular stenosis and small aneurysm. Conclusion Both primary angiitis of the central nervous system and reversible posterior leukoencephalopathy syndrome have certain imaging features, the diagnosis can be correctly made when clinical information is taken into consideration.