中华神经科杂志
中華神經科雜誌
중화신경과잡지
Chinese Journal of Neurology
2012年
10期
702-705
,共4页
孙青%刘明生%崔丽英%邹漳钰%管宇宙%王含%戴毅%钱敏%李本红%杜华%吴双
孫青%劉明生%崔麗英%鄒漳鈺%管宇宙%王含%戴毅%錢敏%李本紅%杜華%吳雙
손청%류명생%최려영%추장옥%관우주%왕함%대의%전민%리본홍%두화%오쌍
米勒·费希尔综合征%脑炎%电生理学%神经节苷脂类%抗体
米勒·費希爾綜閤徵%腦炎%電生理學%神經節苷脂類%抗體
미륵·비희이종합정%뇌염%전생이학%신경절감지류%항체
Miller Fisher syndrome%Encephalitis%Electrophysiology%Gangliosides%Antibodies
目的 总结Miller-Fisher综合征(MFS)和Bickerstaff脑干脑炎(BBE)患者临床及电生理特点,并探索其机制.方法 回顾性分析北京协和医院2000-2011年符合MFS诊断标准的患者(13例)和BBE诊断标准的患者(7例)的病历资料,收集患者临床资料和电生理测定参数,包括感觉和运动神经传导、针极肌电图、F波、皮肤交感反应、脑干听觉诱发电位、瞬目反射等,统计MFS和BBE临床特点和各项电生理检查异常的患者比例.结果 MFS和BBE患者前驱感染以呼吸道症状为主,眼球活动障碍、面瘫、延髓部症状较常见,均有脑脊液蛋白细胞分离,都存在抗GQ1b抗体.但临床上,BBE还有意识障碍等中枢神经系统受累表现.电生理上,MFS和BBE患者感觉神经受累比例分别为6/13、2/7,主要表现为感觉神经动作电位波幅明显下降,感觉神经传导速度轻度减慢或正常；运动神经受累比例分别为2/13、1/7,多表现为运动末端潜伏期轻度延长,复合肌肉动作电位波幅正常；肢体针极肌电图异常比例分别为1/7、0/4；F波出现率异常比例分别为3/13、5/7,部分患者可出现F波出现率明显下降,但可以恢复；皮肤交感反应异常比例分别为1/2、1/3；瞬目反射异常比例分别为1/2、1/1,BBE患者表现为中枢性损害；脑干听觉诱发电位异常比例分别为3/5、1/4,均表现为Ⅰ波潜伏期延长或波幅低.结论 MFS和BBE中枢神经系统和周围神经系统均可受累,但BBE以中枢神经系统受累更常见.MFS和BBE可能是中枢神经系统和周围神经系统受累程度不同的同一种疾病的连续疾病谱.
目的 總結Miller-Fisher綜閤徵(MFS)和Bickerstaff腦榦腦炎(BBE)患者臨床及電生理特點,併探索其機製.方法 迴顧性分析北京協和醫院2000-2011年符閤MFS診斷標準的患者(13例)和BBE診斷標準的患者(7例)的病歷資料,收集患者臨床資料和電生理測定參數,包括感覺和運動神經傳導、針極肌電圖、F波、皮膚交感反應、腦榦聽覺誘髮電位、瞬目反射等,統計MFS和BBE臨床特點和各項電生理檢查異常的患者比例.結果 MFS和BBE患者前驅感染以呼吸道癥狀為主,眼毬活動障礙、麵癱、延髓部癥狀較常見,均有腦脊液蛋白細胞分離,都存在抗GQ1b抗體.但臨床上,BBE還有意識障礙等中樞神經繫統受纍錶現.電生理上,MFS和BBE患者感覺神經受纍比例分彆為6/13、2/7,主要錶現為感覺神經動作電位波幅明顯下降,感覺神經傳導速度輕度減慢或正常；運動神經受纍比例分彆為2/13、1/7,多錶現為運動末耑潛伏期輕度延長,複閤肌肉動作電位波幅正常；肢體針極肌電圖異常比例分彆為1/7、0/4；F波齣現率異常比例分彆為3/13、5/7,部分患者可齣現F波齣現率明顯下降,但可以恢複；皮膚交感反應異常比例分彆為1/2、1/3；瞬目反射異常比例分彆為1/2、1/1,BBE患者錶現為中樞性損害；腦榦聽覺誘髮電位異常比例分彆為3/5、1/4,均錶現為Ⅰ波潛伏期延長或波幅低.結論 MFS和BBE中樞神經繫統和週圍神經繫統均可受纍,但BBE以中樞神經繫統受纍更常見.MFS和BBE可能是中樞神經繫統和週圍神經繫統受纍程度不同的同一種疾病的連續疾病譜.
목적 총결Miller-Fisher종합정(MFS)화Bickerstaff뇌간뇌염(BBE)환자림상급전생리특점,병탐색기궤제.방법 회고성분석북경협화의원2000-2011년부합MFS진단표준적환자(13례)화BBE진단표준적환자(7례)적병력자료,수집환자림상자료화전생리측정삼수,포괄감각화운동신경전도、침겁기전도、F파、피부교감반응、뇌간은각유발전위、순목반사등,통계MFS화BBE림상특점화각항전생리검사이상적환자비례.결과 MFS화BBE환자전구감염이호흡도증상위주,안구활동장애、면탄、연수부증상교상견,균유뇌척액단백세포분리,도존재항GQ1b항체.단림상상,BBE환유의식장애등중추신경계통수루표현.전생리상,MFS화BBE환자감각신경수루비례분별위6/13、2/7,주요표현위감각신경동작전위파폭명현하강,감각신경전도속도경도감만혹정상；운동신경수루비례분별위2/13、1/7,다표현위운동말단잠복기경도연장,복합기육동작전위파폭정상；지체침겁기전도이상비례분별위1/7、0/4；F파출현솔이상비례분별위3/13、5/7,부분환자가출현F파출현솔명현하강,단가이회복；피부교감반응이상비례분별위1/2、1/3；순목반사이상비례분별위1/2、1/1,BBE환자표현위중추성손해；뇌간은각유발전위이상비례분별위3/5、1/4,균표현위Ⅰ파잠복기연장혹파폭저.결론 MFS화BBE중추신경계통화주위신경계통균가수루,단BBE이중추신경계통수루경상견.MFS화BBE가능시중추신경계통화주위신경계통수루정도불동적동일충질병적련속질병보.
Objective To investigate the underlying mechanisms of Miller-Fisher syndrome (MFS) and Bickerstaff' s brainstem encephalitis (BBE) by studying their clinical and electrophysiological characteristics.Methods The clinical and electrophysiological characteristics of 13 MFS and 7 BBE cases in Peking Union Medical College Hospital between 2000 and 2011 were retrospectively analyzed.The electrophysiological parameters included sensory and motor nerve conduction,electromyography,F wave,sympathetic skin response and brainstem auditory evoked potential and blink reflex.Results MFS and BBE had similar clinical characteristics:respiratory symptoms were the most common infectious symptoms before disease onset; Ophthalmoplegia,facial palsy and bulbar symptoms were common; They both had cerebrospinal fluid albuminocytological dissociation and positive serum anti-GQ1b antibody.However,BBE had more central nervous system lesion signs clinically such as conscious disturbance,positive Babinski' s sign and central facial palsy.Electrophysiologically,MFS and BBE also had similar electrophysiological features:sensory nerve abnormality ratios were 6/13,2/7 respectively,with prominently reduced sensory nerve active potential amplitude,normal or slightly slowed sensory conduction velocity; Motor nerves abnormality ratios were 2/13,1/7 respectively,with slightly prolonged distal motor latency and normal compound muscle action potential; Electromyography abnormality ratios were 1/7,0/4 respectively; F wave frequency abnormality ratios were 3/13,5/7 respectively,and in some cases,F wave frequency would restore; Sympathetic skin response abnormality ratios were 1/2,1/3 respectively; Blink reflex abnormalityratios were 1/2,1/1 respectively,with central involvement in BBE; Brainstem auditory evoked potential abnormality ratios were 3/5,1/4 respectively,with wave Ⅰ latency or amplitude abnormality.Conclusion The similarities of clinical and electrophysiological features suggest that MFS and BBE have the same mechanism and they form a continuous spectrum with variable central nervous system and peripheral nervous system involvement.
