以中枢神经症状为首发表现的儿童原发性干燥综合征1例并文献复习  

Childhood primary Sjogren syndrome with central nervous system symptoms as the first manifestation: a case report and literature review

作　　者：黄华 茅幼英 金燕樑 HUANG Hua;MAO Youying;JIN Yanliang(Shanghai Children’s Medical Central Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai 200127, China)

机构地区：[1]上海交通大学医学院附属上海儿童医学中心,上海200127

出　　处：《临床儿科杂志》2017年第10期775-777,共3页Journal of Clinical Pediatrics

摘　　要：目的探讨以中枢神经系统受累为首发表现的儿童原发性干燥综合征(p SS)的诊断和治疗。方法回顾分析1例以发热、头痛起病的11岁男性p SS患儿的临床资料,并复习相关文献。结果患儿经自身抗体、唇腺活检和眼科检查确诊p SS,给予免疫抑制治疗后好转。文献报道,儿童p SS发病率低,其中以神经系统受累为主要表现的更少,而头痛是最常发生的中枢神经症状。糖皮质激素和免疫抑制剂的应用有助于改善病情。结论儿童p SS中枢神经受累症状不典型,自身抗体和唇腺活检有助于诊断。Objective To explore the diagnosis and treatment of childhood primary Sjogren syndrome(pSS)with central nervous system symptoms as the first manifestation.Methods The clinical data of an11-year-old male with pSS which had onset with fever and headache was retrospectively analyzed.The related literatures were reviewed.Results The subject was diagnosed with pSS by autoantibody detection,lip biopsy,and ophthalmologic examination.The symptoms were improved after immunosuppressive therapy.According to the literature,the incidence of childhood pSS was low,in which the incidence with involvment of the nervous system as primanry manifestation was even lower,and headache is the most common symptom of central nervous system.The application of corticosteroids and immunosuppressants may help improve the conditions.Conclusions Involvement of central nervous system in childhood pSS is not typical.Detection of autoantibodies and lip biopsy are helpful for diagnosis.

关 键 词：原发性干燥综合征 中枢神经 儿童 

分 类 号：R725.9[医药卫生—儿科]