新诊断分类在儿童Miller Fisher综合征的临床应用及不同分型临床特征分析

Clinical application of the new classification criteria in children with Miller Fisher syndrome and clinical characteristics analysis of different types of Miller Fisher syndrome

目的了解新诊断分类在儿童Miller Fisher综合征(MFS)的临床应用情况,并描述不完全性、单纯性MFS及重叠综合征的临床特征。方法回顾性分析2015年1月至2019年12月在首都医科大学附属北京儿童医院神经内科住院的MFS患儿诊疗资料,包括人口学特征、临床症状、神经系统查体、辅助检查、诊治方案及预后情况。计数资料用百分比表示,计量资料用中位数表示。结果共23例患儿纳入研究,男14例,女9例,中位年龄4岁8个月。单纯性MFS 3例;不完全性MFS 5例,其中急性眼睑下垂1例,急性共济失调神经病4例;重叠综合征15例,包括MFS/吉兰-巴雷综合征(GBS)13例;MFS/咽颈臂变异型GBS(PCB GBS)及MFS/GBS/Bickerstaff脑干脑炎(BBE)各1例。11例存在除支配眼外肌(Ⅲ、Ⅳ、Ⅵ)之外的颅神经受累,单纯性MFS组2例,MFS/GBS组8例,MFS/GBS/BBE组1例。6例病程中存在自主神经功能障碍。6例病程中出现呼吸肌麻痹,MFS/GBS组5例,MFS/GBS/BBE组1例。Hughes功能分级评分(HG评分)方面,单纯性MFS 3例均为4分;急性眼睑下垂1例为0分;急性共济失调神经病3例为2分,1例为3分;MFS/PCB GBS 1例为3分,MFS/GBS组中,10例为4分,1例为3分,2例为2分;MFS/GBS/BBE 1例为4分。22例患儿接受静脉用免疫球蛋白治疗。出院时所有患儿HG评分不同程度降低,6个月时随访HG评分0分。结论新诊断分类方法在临床中的应用有助于准确诊断不同分型MFS。半数以上MFS会发展为重叠综合征。MFS与GBS或BBE重叠容易出现除支配眼外肌以外的颅神经受累、自主神经障碍及呼吸肌麻痹。患儿病程进展及临床症状存在差异性及可变性,临床诊断应以全面鉴别诊断为前提。所有分型总体预后良好。

Objective To investigate the clinical application of the new classification criteria in children with Miller Fisher syndrome(MFS),and to analyze clinical characteristics of different types of MFS.Methods Clinical data of MFS patients hospitalized in the Department of Neurology,Beijing Children′s Hospital,Capital Medical University from January 2015 to December 2019 were collected and analyzed retrospectively,including demographic characteristics,clinical symptoms,neurological examination findings,laboratory examination data,diagnosis and treatment,and prognosis.The counting data was described by percentage,and the measurement data was described by median.Results A total of 23 patients were included in the research,including 14 males and 9 females,with a median age of 4 years and 8 months.There were 3 cases of pure MFS;5 cases of incomplete MFS,including 1 case of acute ptosis and 4 cases of acute ataxia neuropathy;15 cases of overlapping syndrome,including 13 cases of MFS/Guillain Barre syndrome(GBS),1 case of MFS/pharyngocervical brachial variant GBS(PCB GBS)and 1 case of MFS/GBS/Bickertaff brainstem encephalitis(BBE).In addition toⅢ,ⅣandⅥcranial nerve palsy,11 cases had the involvement of other cra-nial nerves,including 2 cases in pure MFS,8 cases in MFS/GBS and 1 case in MFS/GBS/BBE.Autonomic nervous dysfunction occurred in 6 cases.Respiratory muscle paralysis occurred in 6 cases,including 5 cases in MFS/GBS and 1 case in MFS/GBS/BBE.Graded by the Hughes scoring system(HG score),3 cases with pure MFS were graded 4 points;1 case with acute ptosis was graded 0;3 cases with acute ataxia neuropathy were graded 2 points,and the other one was graded 3 points;1 case with MFS/PCB GBS was graded 3 points;10 cases with MFS/GBS were graded 4 points,1 case was graded 3 points,and the other 2 cases were graded 2 points;1 case with MFS/GBS/BBE was graded 4 points.Twenty-two patients were treated with intravenous immunoglobulin.The HG of all patients at discharge decreased at varying degree,which was graded 0 at 6 months of follow-up.Conclusions The clinical application of the new diagnostic classification method is helpful to the accurate diagnosis of different types of MFS.More than half of MFS cases will develop into the overlapping syndrome.The overlapping of MFS and GBS or BBE is prone to the involvement of cranial nerves except for the external ophthalmic muscles,autonomic nerve dysfunction and respiratory muscle paralysis.The disease course of MFS varies,and its diagnosis should be comprehensively made.All cases of MFS in this study have a satisfactory prognosis.