系统性红斑狼疮伴可逆性后部脑病综合征的临床和MRI特征

Clinical and MRI features of posterior reversible encephalopathy syndrome in patients with systemic lupus erythematosus

目的 分析系统性红斑狼疮（SLE）伴可逆性后部脑病综合征（PRES）的临床和MRI表现,提高对本病的认识和诊疗水平.方法 回顾性分析2008年1月至2013年12月收集的6例经临床证实为SLE伴PRES患者的临床和影像学资料,并复习相关文献.结果 6例中有4例以神经系统症状为首发症状,包括头痛（2例）、癫痫发作（4例）、急性意识模糊（2例）、视物不清（1例）、精神症状（1例）;6例发病时血压不同程度升高;6例均有狼疮性肾炎,其中4例伴有肾功能衰竭;6例发病前均应用激素治疗,其中4例同时应用环磷酰胺治疗.PRES病灶分布：5例顶枕叶受累,额叶4例,颞叶4例,基底节区3例,胼胝体压部1例,小脑半球2例.PRES病灶分布模式：经典型2例,全脑型3例,额上沟型1例.结论 SLE伴PRES患者具有典型的临床和MRI表现,早期认识这类疾病有助于改善治疗效果和预后.

Objective To investigate the clinical and MRI features and pathogenic mechanism of posterior reversible encephalopathy syndrome （PRES） in patients with systemic lupus erythematosus （SLE）.Methods Six cases of PRES in SLE proved by integrated clinical diagnosis were collected from January 2008 to December 2013.The clinical and MRI features of these patients were studied retrospectively and the related literatures were reviewed.Results The initial episode of nervous system was involved in 4 cases.The clinical presentations were headache （2 cases）,seizures （4 cases）,acute confusion state （2 cases）,altered mentation （1 case） and vision change （1 case）.All cases had lupus nephritis and hypertension,in which 4 cases had renal failure;6 cases were treated with immunosuppressive agents and 4 cases with cyclophosphamide for lupus nephritis when they developed PRES.The vasogenic edema lesions were distributed in the parietal or occipital lobe （5 cases）,the frontal lobe （4 cases）,temporal lobe （4 cases）,basal ganglia （3 cases）,splenium （1 case） and cerebellar hemispheres （2 cases）.Three major patterns of PRES included dominant parietal-occipital （2 cases）,the holohemispheric watershed （3 cases）,and superior frontal sulcal （1 case）.Conclusions The clinical and MRI findings of PRES in patients with SLE are typical.Awareness of this entity as early as possible can help to improve curative effect and prognosis.