儿童烟雾病误诊为急性播散性脑脊髓炎原因分析  

Causes of Misdiagnosis of Moyamoya Disease as Acute Disseminated Encephalomyelitis in Children

作　　者：冯源 马洁卉 孙丹[1] 黄胜 胡家胜[1] 吴革菲[1] FENG Yuan;MA Jie-hui;SUN Dan;HUANG Sheng;HU Jia-sheng;WU Ge-fei(Department of Neurology,Wuhan Children's Hospital Wuhan Maternal and Children's Healthcare Hospital Affiliated to Tongji Medical College of Huazhong University of Science&Technology,Wuhan 430014,China)

机构地区：[1]华中科技大学同济医学院附属武汉儿童医院(武汉市妇幼保健院)神经内科,武汉430014

出　　处：《临床误诊误治》2020年第11期20-23,共4页Clinical Misdiagnosis & Mistherapy

基　　金：武汉市卫生计生委科研项目(WX18B06)。

摘　　要：目的探讨儿童烟雾病的诊治要点及误诊为急性播散性脑脊髓炎(acute disseminated encephalomyelitis,ADEM)的原因、防范措施。方法对曾误诊为ADEM的儿童烟雾病2例的临床资料进行回顾性分析。结果本组2例分别因精神反应差伴右侧肢体无力4 d和走路不稳3 d入院,脑病症状明显,初期MRI检查均考虑脱髓鞘改变,皆误诊为ADEM,给予丙种球蛋白和糖皮质激素等治疗,效果不佳。2例分别在出院后6周和病程第15个月再次出现肌无力等局灶性神经系统受损症状,经头颅MRI及磁共振动脉造影(magnetic resonance angiography,MRA)检查确诊为烟雾病,均予脑-硬脑膜-动脉血管融合术治疗。术后3个月随访,2例均恢复良好。结论儿童烟雾病临床表现复杂,可出现脑白质脱髓鞘影像学改变,易误诊为ADEM。总结其临床特点,以尽早诊断及干预,可改善烟雾病患儿脑组织灌注,促进运动功能恢复和提高生活质量。Objective To explore the clinical characteristics,causes of misdiagnosis and preventive measures of moyamoya disease misdiagnosed as acute disseminated encephalomyelitis(ADEM).Methods A retrospective analysis was performed on the clinical data of 2 cases of moyamoya disease misdiagnosed as ADEM.Results One case presented with poor mental response and persistent unilateral limb weakness for 4 d.Another case presented with poor mental response and unstable walking for 3 d.The typical presentation involves encephalopathy.On magnetic resonance imaging(MRI),demyelination changes were considered,and both patients were misdiagnosed as ADEM at the initial stage of treatment.They had a poor response to treatment with gamma globulin and glucocorticoid.During the follow-up,myasthenia or other manifestations of focal neurological deficits reappeared at 6 weeks after discharge and 15 months of the disease course,respectively.They were diagnosed as moyamoya disease by cranial MRI and magnetic resonance angiography(MRA)and treated with brain-dural-arterial vascular fusion.At a follow-up of 3 months,both patients recovered well.Conclusion The manifestations of moyamoya disease were diverse and complex,and the imaging changes of white matter demyelination might occur,which was likely to be misdiagnosed as ADEM.Summarizing its clinical characteristics as well as early diagnosis and intervention can improve brain tissue perfusion,promote the recovery of motor function and improve the quality of life in children with moyamoya disease.

关 键 词：烟雾病 儿童 误诊 脑脊髓炎 急性播散性 

分 类 号：R743[医药卫生—神经病学与精神病学]