儿童Miller-Fisher综合征与Bickerstaff脑炎和吉兰-巴雷综合征重叠的概率和时间

发布时间：2018-04-03 18:22

  本文选题：Miller-Fisher综合征　切入点：Bickerstaff脑炎　出处：《临床儿科杂志》2017年06期

【摘要】：目的探讨儿童Miller-Fisher综合征(MFS)与Bickerstaff脑炎(BBE)、典型吉兰-巴雷综合征(GBS),吉兰-巴雷变异型即咽-颈-臂吉兰-巴雷综合征(PCB-GBS)重叠的概率和时间。方法回顾分析确诊的128例MFS患儿的临床资料。结果 128例MFS患儿中,60例为单纯MFS(眼肌麻痹、共济失调、反射减弱或消失,不伴有肢体无力和嗜睡,实验室检查提示有脑脊液蛋白-细胞分离和/或血清抗-GQ1b抗体阳性),28例发展为MFS/PCB-GBS(符合MFS诊断标准,并伴有咽部、颈部和上肢无力,上肢反射减弱或消失,不伴下肢无力),22例发展为MFS/GBS(符合MFS诊断标准,并伴有四肢无力),18例发展为MFS/BBE(符合MFS诊断标准,并伴有嗜睡、椎体束征阳性)。四组患儿发病年龄、起病至开始治疗时间、Hughes功能分级评分以及有前驱感染病史、血清抗-GQ1b抗体阳性比例、脑脊液蛋白-细胞分离比例均无统计学意义(P0.05)。MFS患儿发展为BBE、PCB-GBS、GBS的时间在起病后10天内。结论 MFS患儿重叠PCB-GBS、BBE、GBS的概率为50%,一般发生在症状起始后10天内,临床医师需要注意时间窗,合理调整用药。
[Abstract]:Objective to investigate the probability and time of overlap between Miller-Fisher syndrome in children and Bickerstaff encephalitis, typical Guillain-Barre syndrome and Guillain-Barre syndrome.Methods the clinical data of 128 children with MFS were retrospectively analyzed.Results among the 128 cases of MFS, 60 cases were simple MFSs (ophthalmoplegia, ataxia, weakening or disappearance of reflex, no limb weakness and somnolence).Laboratory examination showed that 28 patients with cerebrospinal fluid protein-cell separation and / or serum anti-GQ1b antibody positive had developed into MFS / PCB-GBS (in accordance with MFS diagnostic criteria, accompanied by weakness of pharynx, neck and upper limb, weakening or disappearance of upper limb reflex).Twenty-two patients without lower limb weakness developed into MFS / GBS (in accordance with MFS diagnostic criteria and 18 with limb weakness) (MFS diagnostic criteria, somnolence and spinal tract sign positive).In the four groups, the age of onset, the time from the onset of the disease to the beginning of treatment, the score of Hughes functional grading, the history of pre-infection, and the positive rate of anti-GQ1b antibody in serum were observed in the four groups.Conclusion the probability of overlapping PCB-GBS BBEBS in children with MFS is 50, which usually occurs within 10 days after the onset of symptoms. Clinicians should pay attention to the time window and adjust the use of drugs reasonably.
【作者单位】： 华中科技大学同济医学院附属武汉儿童医院;湖北省新华医院神经内科;
【基金】：武汉市科技创新平台——儿童神经疾病临床医学研究中心资助项目(No.武科计2014-160号)
【分类号】：R745.43
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本文编号：1706373