首页> 中文期刊> 《中国神经免疫学和神经病学杂志》 >Miller-Fisher综合征与Guillain-Barré 综合征r非核心临床表现 、电生理及脑脊液比较

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

         

摘要

According to the new diagnostic criteria in 2014 ,classical Guillain-Barré syndrome (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 M FS 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<0.05 ; (1144.46 ± 764.31) mg/L vs. (691.79 ± 469.35) mg/L ;t= -3.371 , P< 0.01] . Conclusions The rate of patients whose peak Hughes scores≥3 in classical GBS patients is higher than MFS patients , The degree of disability is more serious in classical GBS. Non-core clinical features , electrophysiological and CSF tests besides the accepted specific core clinical features have obvious difference between these two subtypes ,all these can contribute to understanding of the new diagnostic criteria ,which can help to diagnose and identify both.%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M FS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M FS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M FS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%),M FS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%),M FS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M FS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M FS者更多,残疾更重,需要尽早准确鉴别.除公认的GBS与M FS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.

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