儿童狼疮性脑病19例临床分析  

Clinical Analysis of 19 Children with Neuropsychiatric Systemic Lupus Erythematosus

作　　者：陈名华[1] 赖宽[1] 彭宇声 张静[1] 熊浩[1] 王智琴[1] 曾抗[1] 

机构地区：[1]南方医科大学南方医院皮肤科,广东广州510515 [2]武警广东省总队医院皮肤科,广东广州510507

出　　处：《皮肤性病诊疗学杂志》2016年第1期13-17,共5页Journal of Diagnosis and Therapy on Dermato-venereology

基　　金：国家自然科学基金(编号:81301371);广东省自然科学基金(编号:2014A030313350)

摘　　要：目的:观察儿童狼疮性脑病(NPSLE)的临床特点。方法:收集总结我院19例NPSLE患儿的临床资料(病史、查体、辅助检查、治疗等)进行分析。结果:7例患儿首诊时以神经精神症状为主要表现,12例患儿出现NPSLE前有不同病程的SLE病史。NPSLE患儿中癫痫症状发生频率最高(7例)。均出现狼疮活动的指标(如血沉加快,补体C3、C4下降)。6例患儿行头颅MRI检查,均有阳性发现。大剂量糖皮质激素、丙种球蛋白冲击联合免疫抑制剂以及血液净化等综合治疗为NPSLE有效治疗方法。结论:NPSLE可发生于儿童。癫痫在NPSLE患儿中发生率高,是SLE致死主要原因之一。脑脊液(CSF)、电子计算机断层扫描(CT)、磁共振(MRI)、脑电图(EEG)等联合检查有助于NPSLE的早期诊断。对NPSLE应采取个体化综合治疗。Objective： To investigate the clinical characteristics of Neuropsychiatric Systemic Lupus Erythematosus（ NPSLE） in children and review related articles. Methods： 19 cases of NPSLE in children who were in our hospital during the period of December 2004 to June 2015 were reviewed and analyzed. Results： 7 cases of children admitted to our hospital showed neuropsychiatric（ NP） symptoms as the main manifestation,and 12 cases developed into neuropsychiatric lupus after different courses of SLE. Seizures are the most frequent NP symptom in NPSLE children（ 7 cases）. All patients exhibited activity markers such as increased erythrocyte sedimentation rate（ ESR）,decreased complement C3 and complement C4. All 6 cases showed abnormal result for cranial MRI scan. Usually large-dose IV Glucocorticoid,IV immunoglobulin and immunosuppressive agent combined with hemopurification was shown to be effective. Conclusion：NPSLE can occur in children with a high incidence of seizures which may be one of the leading causes of death. It is necessary to combine CSF、CT、MRI and EEG examination for early diagnosis of NPSLE. Individual comprehensive therapy should be given accordingly for NPSLE.

关 键 词：系统性红斑狼疮 狼疮性脑病 儿童 治疗 

分 类 号：R725.9[医药卫生—儿科]