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

摘要

Objective To investigate the probability and timing of childhood Miller-Fisher syndrome (MFS) progressing to Bickerstaff brainstem encephalitis (BBE), classical Guillain-Barre syndrome (GBS), and pharyngeal-cervical-brachial (PCB-GBS). Methods The clinical data of 128 children with confirmed MFS diagnosis were retrospectively analyzed. Results Among 128 children, 60 cases were simple MFS (ocular muscle paralysis, ataxia, reflexes diminished or disappeared, without limbs weakness and lethargy; laboratory tests suggest cerebrospinal fluid protein-cell separation and/or serum anti-GQ1b antibody positive), 28 cases developed MFS/PCB-GBS (met MFS diagnosis criteria, accompanied by weakness of pharynx, neck and upper limb, weakened or disappeared of upper limb reflex, without weakness of lower limb), 22 cases developed MFS/GBS (met MFS diagnosis criteria, accompanied by weakness of limb), 18 cases developed MFS/BBE (met MFS diagnosis criteria, accompanied by lethargy, pyramidal tract positive). There were no differences in the age at onset, the interval from onset to the start of the treatment, Hughes functional grading, and the percentage of cases having a history of preceding infections, the rate of positive serum anti-GQ1b antibody, the ratio of albumin cytological dissociation in cerebrospinal fluid among 4 groups (P>0.05). The interval from MFS onset to progression to MFS/PCB-GBS, MFS/GBS, or MFS/BBE was within 10 days. Conclusions In children with MFS, 50% developed PCB-GBS, GBS, or BBE, which occurred within 10 days after onset. Clinicians should pay attention to the time window and adjust the medicine rationally.%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(P>0.05).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.