摘要
目的探讨急性播散性脑脊髓炎(ADEM)的临床表现及神经影像学异常恢复情况,以提高对ADEM的认识。方法对苏州大学附属儿童医院神经内科收治的47例ADEM患儿随访1年,分析其临床表现、辅助检查(特别是神经影像学检查)、治疗方法及预后转归等资料,总结其临床及神经影像学异常的恢复情况。结果47例患儿中,37例(78.72%)有前驱感染史,5例(10.64%)有疫苗接种史;患儿临床表现多样,包括躯体不适36例(76.60%),发热30例(63.83%),意识障碍30例(63.83%),癫痫发作28例(59.57%),头痛、头晕24例(51.06%),恶心、呕吐22例(46.81%),运动障碍15例(31.91%),颅神经损伤15例(31.91%),共济失调9例(19.14%),精神行为异常8例(17.02%)。头颅磁共振成像(MRI)显示病灶呈现多发、不对称的斑片状或大片状信号影,7例(14.89%)累及脊髓。所有病例采用甲泼尼龙[15—20In((kg·d)]联合静脉丙种球蛋白(总量2g/kg,分3—5d用完)治疗,甲泼尼龙3d后逐渐减量,最后改为泼尼松[1.0—2.0mg/(kg·d)]口服,总疗程2~3个月。随访47例ADEM患儿中,0.5个月时所有患儿MRI病灶较前缩小,其中4例(8.51%)病灶完全消失;1个月时13例(27.66%)病灶完全消失;3个月时23例(48.94%)病灶完全消失;6个月时32例(68.09%)病灶完全消失;1年时43例(91.49%)病灶完全消失,余4例(8.51%)有后遗病灶。结论儿童ADEM的临床表现多样,大部分预后良好。68.09%的病例可在发病6个月影像学病灶完全消失,91.49%可在发病1年影像学病灶完全消失,仅少部分遗留长期病灶。
Objective To explore the clinical manifestations and recovery from neuroimaging abnormalities of acute disseminated encephalomyelitis(ADEM),in order to improve the understanding of ADEM.Methods A total of 47 children with ADEM in Department of Neurology,Children's Hospital of Soochow University were followed up for 1 year.The clinical manifestations,auxiliary examination(especially neuroimaging),treatment and prognosis were analyzed.The recovery conditions from clinical and neuroimaging abnormalities were summarized.Results Among 47 cases,37 cases(78.72%)had prodromic infection history,and 5 cases(10.64%)had the history of vaccination.The clinical manifestations were varied,including 36 cases(76.60%)with somatic discomfort,30 cases(63.83%)with fever,30 cases(63.83%)with disturbance of consciousness,28 cases(59.57%)with epileptic seizure,24 cases(51.06%)with headache and dizziness,22 cases(46.81%)with nausea and vomiting,15 cases(31.91%)with dyskinesia,15 cases(31.91%)with cranial nerve injury,9 cases(19.14%)with incoordination,and 8 cases(17.02%)with mental and behavior disorders.Abnormal lesions presented multiple,asymmetric patchy and large patchy signal image showed by brain magnetic resonance imaging(MRI),and 7 cases(14.89%)involved the spinal cord.All patients received the treatment of Methylprednisolone[15-20 mg/(kg·d)combined with intravenous gamma globulin(total 2 g/kg,3 to 5 days).The dose of Methylprednisolone gradually decreased after it had been used for 3 days.In the end,it was replaced by an oral administration of Prednisone[1.0-2.0 rag/(kg·d)],and the total treatment period was for 2-3 months.The follow-ups of 47 children with ADEM showed that the MRI lesions of all the children were reduced and the MRI lesions in 4(8.51%)of them disappeared completely after 0.5 month of follow-up;the lesions disappeared completely in 13 cases(27.66%)after l month of follow-up;the lesions disappeared completely in 23 cases(48.94%)after 3 months of follow-up;the lesions disappeared completely in 32 cases(68.09%)after 6 months of follow-up;the lesions disappeared completely in 43 cases(91.49%)after 1 year of follow-up;and finally there were 4 cases(8.51%)with residual lesions.Conclusion The clinical manifestations of children with ADEM are varied.Most children with ADEM have a faw)rable prognosis,hnaging lesions of 68.09%of the cases completely disappeared after the onset of 6 months.Imaging lesions in 91.49%of the cases completely disa-ppeared at the onset of 1 year.Only a small fraction of patients present permanent lesions.
作者
徐敏
汤继宏
师晓燕
张利亚
徐巧岚
Xu Min;Tang Jihong;Shi Xiaoyan;Zhang Liya;Xu Qiaolan(Department of Neurology,Children's Hospital Affiliated to Soochow University,Suzhou 215011,Jiangsu Province,China;Department of Pediatrics,the Third People's Hospital of Yancheng,Yancheng 224001,Jiangsu Province,China)
出处
《中华实用儿科临床杂志》
CSCD
北大核心
2017年第12期936-939,共4页
Chinese Journal of Applied Clinical Pediatrics
基金
国家自然科学基金青年基金(81301129)
关键词
急性播散性脑脊髓炎
临床特征
神经影像学
磁共振成像
儿童
Acute disseminated encephalomyelitis
Clinical manifestation
Neuroimaging
Magnetic resonance
