Miller-Fisher综合征与Guillain-Barré综合征非核心临床表现、电生理及脑脊液比较  

The comparison of non-core clinical features,electromyography and cerebrospinal fluid test in Miller-Fisher syndrome and Guillain-Barré syndrome

作　　者：朱莹 周夏俊 管阳太[2] ZHU Ying;ZHOU Xiajun;GUAN Yangtai(Department of Neurology,Renji Hospital,Jiaotong University,Shanghai 200127,China)

机构地区：[1]上海国际医学中心神经内科,201314 [2]上海交通大学医学院附属仁济医院神经内科,200127

出　　处：《中国神经免疫学和神经病学杂志》2018年第1期22-25,31,共5页Chinese Journal of Neuroimmunology and Neurology

摘　　要：目的 2014年新的GBS谱系疾病诊断标准提出,典型吉兰-巴雷综合征(Guillain-Barrésyndrome,GBS)与Miller-Fisher综合征(Miller-Fisher syndrome,MFS)为同一疾病谱的不同亚型,主要根据各自特殊的核心临床表现进行诊断和鉴别诊断。本研究分析典型GBS与MFS患者的非核心临床表现、神经电生理和脑脊液检测结果的差异,以期更好地鉴别和认识GBS谱系疾病中这两大主要亚型。方法回顾分析上海交通大学医学院附属仁济医院2013-01—2017-03收住的MFS患者37例,典型GBS患者79例。比较两组患者的非核心临床表现、神经电生理及脑脊液检查结果的差异。结果 (1)临床表现:1)前驱感染方面:MFS患者呼吸道感染发生率高于GBS患者(48.6%比22.8%,P<0.05),而GBS患者腹泻发生率高于MFS组(34.1%比2.7%,P<0.01);2)非核心症状方面:MFS组浅感觉障碍出现率较GBS组低(37.8%比60.8%,P<0.05);3)高峰期Hughes评分:MFS组评分≥3分者较GBS组少(8.1%比75.9%,P<0.01)。(2)电生理检查:典型GBS组主要表现为运动伴感觉神经传导异常(54.4%)或单纯运动神经传导异常(27.8%),MFS组主要表现为单纯H反射异常(35.1%)、单纯感觉传导异常(35.1%)和正常肌电图(13.5%);典型GBS组F波异常多见(82.3%),MFS组出现F波异常少见(5.4%)。(3)脑脊液表现:典型GBS组脑脊液蛋白细胞分离率和蛋白水平均高于MFS组[82.4%比57.1%,P<0.05;(1144.46±764.31)mg/L比(691.79±469.35)mg/L,P<0.05]。结论高峰期GBS患者Hughes评分≥3分者较MFS者更多,残疾更重,需要尽早准确鉴别。除公认的GBS与MFS特殊的核心临床表现外,两者的非核心临床表现、脑脊液和电生理检查结果均存在差异,认识这些差异有利于更好地理解新的诊断标准,也有助于两者的诊断和鉴别。According to the new diagnostic criteria in 2014,classical Guillain-Barrésyndrome(GBS)and Miller-Fisher syndrome(MFS)belong to GBS spectrum,their diagnosis and differential diagnoses depends largely on specific core clinical features.This research compared the difference between classical GBS and MFS in non-core clinical features,nerve conduction studies and cerebrospinal fluid(CSF)tests.Methods We retrospectively reviewed results of non-core clinical features,nerve conduction studies and CSF tests of 37 MFS patients and compared them with 79 classical GBS patients in Renji Hospital from Jan 2013 to Mar 2017.Results(1)Non-core clinical features:1)Precursor infection:the MFS group had higher rates of precursor respiratory tract infection than the classical GBS group(48.6%vs.22.8%,P<0.01),however the classical GBS group had higher rates of precursor diarrhea than the MFS group(34.1%vs.2.7%,P<0.05),both reached statistical significance. 2)Non-core symptoms:The patients in the MFS group had lower rates of paresthesia than the patients in the classical GBS group(37.8%vs.60.8%,P<0.05).3)The Hughes functional grading scale scores(Hughes scores)at the peak of disease:The rate of patients whose Hughes scores≥3(suggest poor outcomes)in the MFS group was lower than the classical GBS group(8.1%vs.75.9%,P<0.01).(2)Electromyography:1)The major nerve conduction findings in patients with classical GBS were motor and sensory conduction abnormality(54.4%)or motor conduction abnormality(27.8%).The major nerve conduction findings in patients with MFS were absent or reduced H-reflexes(approximately 35.1%),sensory conduction abnormality(approximately 35.1%)or normal nerve conduction findings(approximately 13.5%),P<0.05.2)The F wave abnormality were hardly observed in MFS patients(5.4%),however usually observed in classical GBS patients(82.3%),P<0.05.(3)Examination of CSF:the classical GBS group(82.4%)had higher rates of albuminocytologic dissociation and higher average CSF protein level than the MFS group[82.4%vs.57.1%;χ2=5.022,P<

关 键 词：MILLER-FISHER综合征 吉兰-巴雷综合征 神经传导检查 脑脊液 Hughes评分 

分 类 号：R744.5[医药卫生—神经病学与精神病学]