摘要：
目的 总结系统性红斑狼疮与肾病综合征引起的可逆性后部白质脑病综合征临床及影像学特征。方法 选择2013年1月至2017年12月共53例确诊为可逆性后部白质脑病综合征的患者，原发病分别为系统性红斑狼疮（SLE组，31例）或肾病综合征（NS组，22例），通过不同序列MRI检查方法， 观察不同病因所致可逆性后部白质脑病综合征的影像学表现、受累部位、病变严重程度。结果 NS组癫痫发作（χ2=9.255， P=0.002）、视觉障碍（χ2=7.005， P=0.008）发生率高于SLE组；而SLE组则以双侧侧脑室旁（校正χ2=5.377， P=0.020）、基底节区（校正χ2=4.825， P=0.028）和半卵圆中心（χ2=8.113， P= 0.004）受累为主；两组影像学分型差异具有统计学意义（Fisher确切概率法： P=0.029），NS组顶枕叶型分布所占构成比明显高于SLE组（Fisher确切概率法： P=0.016）；与NS组相比，经药物治疗后SLE组患者预后更佳、病程更短（t=2.612， P=0.013）。结论 系统性红斑狼疮和肾病综合征所引起的可逆性后部白质脑病综合征在影像学表现上存在一定差异，对理解该病病理生理学机制、明确诊断具有鉴别意义。
关键词:
后部白质脑病综合征,
红斑狼疮, 系统性,
肾病综合征,
磁共振成像
Abstract:
Objective To summarize clinical and imaging features of posterior reversible leukoencephalopathy syndrome (PRES) caused by systemic lupus erythematosus (SLE) and nephrotic syndrome (NS). Methods A total of 53 patients clinically diagnosed with PRES from January 2013 to December 2017 were selected. The patients were divided into SLE group (31 patients) and NS group (22 patients) according to their primary diseases. Differences between the 2 groups in imaging features, affected sites and severity of lesions were compared. Results Incidence rates of epileptiform seizure (χ2 = 9.255, P = 0.002) and visual impairment (χ2 = 7.005, P = 0.008) of the NS group were higher than those in the SLE group. In the SLE group, bilateral periventricular areas (adjusted χ2 = 5.377, P = 0.020), basal ganglia(adjusted χ2 = 4.825, P = 0.028) and centrum semiovale (χ2 = 8.113, P = 0.004) were mainly involved. The differences of imaging type between the 2 groups were statistically significant (Fisher's exact probability: P =0.029). The proportion of parietal occipital lobe distribution in NS group was significantly higher than that in SLE group (Fisher's exact probability: P = 0.016). After treatment, PRES recovery in the SLE group was better and faster than that in NS group (t = 2.612, P = 0.013). Conclusions Common pathogens of PRES namely SLE and NS are different in certain degree of imaging features, and this will be significant to understand pathophysiological mechanism and clinical diagnosis of this disease.
Key words:
Posterior leukoencephalopathy syndrome,
Lupus erythematosus, systemic,
Nephrotic syndrome,
Magnetic resonance imaging
金鑫, 李建瑞, 陆梦洁, 王兴东, 李德龙, 张志强. 系统性红斑狼疮与肾病综合征致可逆性后部白质脑病综合征临床及影像学特征分析[J]. 中国现代神经疾病杂志, 2019, 19(9): 688-692.
JIN Xin, LI Jian-rui, LU Meng-jie, WANG Xing-dong, LI De-long, ZHANG Zhi-qiang. Clinical and imaging features of the posterior reversible leukoencephalopathy syndrome in different causes of systemic lupus erythematosus and nephrotic syndrome[J]. Chinese Journal of Contemporary Neurology and Neurosurgery, 2019, 19(9): 688-692.