儿童神经白塞综合征5例的临床特征及预后分析  

Analysis of the clinical features and prognosis of neuro-Behcet′s syndrome in 5 children

作　　者：王莲[1] 闫淯淳 王艺霖 马立燕[1] 唐永霞 赖建铭[4] Wang Lian;Yan Yuchun;Wang Yilin;Ma Liyan;Tang Yongxia;Lai Jianming(Department of Pediatrics,General Hospital of Ningxia Medical University,Yinchuan750004,China;Department of Radiology,Children′s Hospital Affiliated to the Capital Institute of Pediatrics,Beijing100020,China;Department of Radiology,General Hospital of Ningxia Medical University,Yinchuan750004,China;Department of Rheumatology and Immunology,Children′s Hospital Affiliated to Capital Institute of Pediatrics,Beijing100020,China)

机构地区：[1]宁夏医科大学总医院儿科,银川750004 [2]首都儿科研究所附属儿童医院放射科,北京100020 [3]宁夏医科大学总医院放射科,银川750004 [4]首都儿科研究所附属儿童医院风湿免疫科,北京100020

出　　处：《中华儿科杂志》2025年第1期80-83,共4页Chinese Journal of Pediatrics

基　　金：宁夏回族自治区重点研发计划(2023BEG03004)。

摘　　要：目的探讨儿童神经白塞综合征(NBS)的临床特征及预后。方法病例系列研究,分析2014年4月至2024年4月宁夏医科大学总医院儿科、首都儿科研究所附属儿童医院风湿科确诊的5例NBS患儿的临床、脑磁共振成像、实验室及随访资料,评估患儿NBS的治疗效果。结果5例NBS患儿中男2例、女3例,就诊年龄8~17岁,发病到诊断时间为2 d至4年。2例患儿治疗期间出现头晕、头痛、抽搐,1例患儿出现意识障碍,1例患白塞综合征4年后逐渐出现失语、肢体活动障碍、吞咽困难、肌力下降,1例无临床症状。4例患儿C反应蛋白、红细胞沉降率均增高,1例患儿脑脊液白细胞、免疫球蛋白G增高。4例患儿脑磁共振成像表现为多部位病变,为双侧额叶、枕叶、顶叶、侧脑室旁以及胼胝体病变;1例脑磁共振成像示多发脱髓鞘病,颈胸椎磁共振成像示颈髓及胸髓纤细。5例患儿均使用激素联合免疫抑制剂或生物制剂治疗。随访6个月至4年,4例治疗效果良好,1例最终放弃治疗。结论NBS临床表现无特异性,脑磁共振成像提示病变累及部位和病变形态无特异性。激素联合免疫抑制剂或生物制剂治疗NBS预后良好。ObjectiveTo investigate the clinical features and prognosis of neuro-Behçet′s syndrome(NBS)in children.MethodThe clinical,brain magnetic resonance imaging and laboratory data of 5 children with NBS diagnosed in the Department of Pediatrics,General Hospital of Ningxia Medical University and Department of Rheumatology and Immunology,Children′s Hospital Affiliated to Capital Institute of Pediatrics from April 2014 to April 2024 were analyzed retrospectively.The follow-up method was retrospective outpatient or inpatient visit to evaluate the treatment effect of NBS.ResultAmong the 5 NBS cases,2 were male and 3 were female.The age of admission ranged from 8 to 17 years,the time from onset to diagnosis was 2 days to 4 years.Two patients had dizziness,headache and convulsions during the treatment of NBS,1 patient had disturbance of consciousness,1 patient gradually developed aphasia,limb movement disorder,dysphagia and muscle weakness after 4 years of Behçet's syndrome,and 1 patient had no clinical symptoms.C-reactive protein and erythrocyte sedimentation rate were increased in 4 cases,and cerebrospinal fluid white blood cells and immunoglobulin G were increased in 1 case.Brain magnetic resonance imaging of 4 children showed multiple lesions,including bilateral frontal lobe,occipital lobe,parietal lobe,periventricular and corpus callosum lesions.Brain magnetic resonance imaging showed multiple demyelinating diseases in 1 case,and cervical and thoracic magnetic resonance imaging showed slender cervical and thoracic spinal cord.All patients were treated with corticosteroids combined with immunosuppressants or biological agents.The children were followed up for 6 months to 4 years,and 4 cases had good treatment results,and 1 case finally gave up treatment.ConclusionsThe clinical manifestations of NBS are not specific,and brain magnetic resonance imaging shows that the lesion location and morphology are not specific.NBS children treated with corticosteroids combined with immunosuppressive agents or biological agents h

关 键 词：儿童 神经 白塞综合征 磁共振成像 

分 类 号：R725.9[医药卫生—儿科]