原发性中枢神经系统血管炎2例MRI表现并文献复习

MRI appearances of primary angiitis of the central nervous system:2 cases report and literature review

目的:报告2例原发性中枢神经系统血管炎(PACNS)病例MRI影像学表现,结合文献分析,提高对PACNS影像学特点的认识。方法:回顾性总结本院2例PACNS病例临床及MRI资料,并汇总分析文献报告的PACNS影像资料。结果:病例1,男性,35岁,以语言、认知功能障碍为主诉;病例2,女性,42岁,以癫痫发作为主诉。2例MRI平扫表现为双侧大脑半球皮质、白质多发长T1、T2信号灶,增强扫描其内可见点状、片状及脑回状强化;磁敏感加权成像(SWI)提示脑内多发微出血灶,1例磁共振波谱成像(MRS)病灶区N-乙酰天门冬氨酸(NAA)减低、可见乳酸峰,灌注成像(PWI)病灶区域为低灌注,扩散张量成像(DTI)显示病变区域白质纤维束明显减少,血管壁高分辨成像,病灶附近小血管壁环状增厚并均匀强化。结论:典型PACNS的MRI影像学表现为大脑半球皮质及皮质下区多发长T1、长T2信号病灶,呈点状、脑回状强化,中小血管管壁增厚并强化,常见多发微出血灶。多模态MRI成像有助于PACNS诊断。

Objective: To analysis the clinical and imaging features of 2 cases of primary angiitis of central nervous system(PACNS), and to review the literature. Methods: PACNS cases in Zhongnan Hospital of Wuhan University were collected, then, the cases were retrospectively studied by describing and summarizing its clinical manifestations and imaging features. Results: Two cases were collected. Case1 was male, aged 35 years, with language and cognitive dysfunction as his initial symptoms; Case 2 was female, aged 42 years, complained with seizures. Both patients received brain MRI scan, and showed bilateral lesions in cortex and subcortical regions. Enhanced MRI scan showed spot, patchy and cerebriform enhancements, while multiple micro-hemorrhages were detected by susceptibility weighted imaging(SWI) in both cases. Diffusion weighted imaging(DWI) found no diffusion limited.By magnetic resonance spectroscopy(MRS), one case showed N-acetyl aspartate(NAA) peak decrease, slightly increased choline(Cho) peak, and lactic acid(La) peak. Decrease of perfusion and whiter matter fibers were showed by perfusion weighted imaging(PWI) and diffusion tensor imaging(DTI),respectively. High resolution MRI of vessel was performed to show the ring thickening and enhancement of small vessel inside the lesion. Conclusion: Clinical diagnosis of PACNS is difficult.MRI is an important examination method, the new technique such as multimodality MRI and HR MRI should be consummated to improve the understanding of the disease and the diagnostic accuracy.