儿童狼疮性脑病临床特征

Clinical features of neuropsychiatric systemic lupus erythematosus in children

目的探讨儿童狼疮性脑病(NPSLE)的临床特点、辅助检查及治疗。方法对11例NPSLE患儿的临床资料进行分析。结果11例NPSLE患儿均有不同程度的头痛症状,意识障碍及失语各1例,抽搐、记忆力减退和共济失调各3例,出现病理反射及脑膜刺激征各4例。血清学均呈狼疮活动表现。3例脑脊液常规无异常,寡克隆抗体明显升高。其中10例行头颅CT检查,异常9例;10例患儿行脑电图检查,正常1例,且与CT无交叉。经个体化综合治疗,11例患儿神经精神症状均有不同程度的改善。结论系统性红斑狼疮累及神经系统症状最常表现为头痛。目前NPSLE主要根据临床表现作出诊断,血清学、影像学及脑电图检查有助于狼疮性脑病的诊断。对NPSLE的治疗强调个体化的综合治疗。

Objectives To analyze the clinical features, auxiliary examinations and treatment of neuropsychiatric systemic lupus erythematosus （NPSLE） in children, Methods The clinical data of 11 children with NPSLE were reviewed from January, 2002 to September, 2006. Results All of 11 patients had headache with different degree, other symptoms included consciousness disturbance and aphasia （one case each）, convulsion, decreased memory and ataxia （3 cases each）, positive pathologic reflex and meningeal irritation sign （4 cases each） , All showed active SLE on orrhology examination, Nine of 10 cases accepted cranial CT scan were abnormal. One of the 10 cases taken EEG was normal with normal CT finding. The neuropsychiatric symptoms in 11 patients were improved within short term ＇after individualized combined therapy. Conclusions Headache is the most common symptom of SLE involving with neuropsychiatric system. Currently, the diagnosis of NPSLE is made mainly based on the clinical manifestations. However, orrhology, imaging and neurophysiology examinations can be helpful for the confirmation of NPSLE. Individualized combined therapy for NPSLE should be emphasized.