系统性红斑狼疮合并可逆性后部脑病综合征1例  

Posterior reversible encephalopathy syndrome in systemic lupus erythematosus: a case report

作　　者：张晓慧[1] 邓雪蓉[1] 李凡[2] 朱颖[3] 张卓莉[1] ZHANG Xiao-hui;DENG Xue-rong;Li Fan;ZHU Ying;ZHANG Zhuo-li(Department of Rheumatology and Immunology;Department of Neurology;Department of Radiology,Peking University First Hospital,Beijing 100034,China)

机构地区：[1]北京大学第一医院风湿免疫科,北京100034 [2]北京大学第一医院神经内科,北京100034 [3]北京大学第一医院影像科,北京100034

出　　处：《北京大学学报（医学版）》2018年第6期1102-1107,共6页Journal of Peking University:Health Sciences

摘　　要：系统性红斑狼疮（systemic lupus erythematosus,SLE）是风湿免疫科常见的一种多系统受累的自身免疫病。神经系统症状在SLE患者中并不少见,发生率为21%～95%,但仅有13%～38%归因于SLE本身。除神经精神狼疮（neuropsychiatric systemic lupus erythematosus,NPSLE）外,药物、感染、代谢性因素等亦可引发精神神经系统异常,此外,可逆性后部脑病综合征（posterior reversible encephalopathy syndrome,PRES）也不容忽视[1]。本文就1例SLE合并PRES的病例来分析当狼疮患者出现神经精神表现时的诊断及鉴别诊断思路,并通过文献复习.This case report concerns a 22-year-old woman who had been diagnosed with systemic lupus erythematosus（ SLE）. She had intermittent fever,butterfly erythema,photosensitivity,oral ulcers,and multiple arthralgia in the past seven years,but she did not adhere to regular treatments. The edema of the lower extremities and face aggravated in the recent two weeks,so she was admitted to our Department of Rheumatology and Clinical Immunology. Meanwhile,we found she had severe hypertension,the maximal blood pressure was 170/120 mm Hg. The patient had high SLE disease activity（ the disease activity index score was as high as 23） with blood involvement,acute renal insufficiency,multiple serous effusion and rash. After one week treatments of intravenous methylprednisolone 80 mg daily and other drugs,her conditions made some extent improvement. However,she suffered sudden epileptic attacks. No positive neuro-pathological signs were found,and the blood pressure was up to 190/130 mm Hg before the onset of the seizures. Her cerebrospinal fluid（ CSF） pressure was 330 mm H2 O,the CSF protein level was normal value,and the white blood cell count was 0 cell/mm3,with no signs of infection. Cranial MRI showed vasogenic edema at bilateral parietal,occipito-parietal regions,and centrum ovale. We prescribed drugs of decreasing intracranial pressure,intravenous drugs of decreasing blood pressure and midazolam for sedation,without corticosteroid impulse therapy. She recovered consciousness in the next day,without epilepsy recurrence. We eventually diagnosed it as posterior reversible encephalopathy syndrome（ PRES）,according to the history,laboratory results,imaging featuresand clinical outcome. PRES is a disorder of reversible subcortical vasogenic brain edema in patients with acute neurological symptoms（ eg,seizures,encephalopathy,headache,and visual disturbances）. PRES is mainly caused by blood pressure changes or endothelial injury,which lead to breakdown of the blood-brain barrier and subsequent brain edema.Most patie

关 键 词：红斑狼疮 癫痫 脑病 神经精神狼疮 

分 类 号：R593.241[医药卫生—内科学]