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Guillain-Barre´syndrome(GBS)•AIDP•AMAN•AMSAN•MFSGanglioside•神经节苷脂广泛分布于全身各组织细胞的外表面,在神经系统中含量丰富,在周围神经系统中主要存在于轴索，而在胶质和髓鞘中浓度较低•根据

其糖基结合唾液酸分子的数目及部位分为GM、GD、GT及GQPathology•AMANandAMASNareassociatedwithCampylobacterjejunienteritisandantibodiesagainstgangliosideschemicalstru

ctureofgangliosides不同种类的神经节苷脂在周围神经系统的分布具有各自不同的特点•GM1除广泛分布于周围神经的轴索，还分布于郎飞氏节及施万细胞近轴索侧细胞膜，在运动神经髓鞘内GM1的含量多于其在感觉神经髓鞘内的含量•GD1b在后根神经节大感觉神经元分布较多•GQ

1b在动眼、滑车、外展神经的髓鞘中含量高于其他颅神经特定抗神经节苷脂抗体和具有某些特异临床特点的神经系统疾病相关•抗GM1抗体见于急性运动轴索性神经病（AMAN）及脱髓鞘性GBS，IgM类GM1抗体见于多灶性运动神经病（MMN），症状学研究提

示GM1抗体阳性的患者更多出现肢体无力，而感觉障碍相对较少•抗GD1b抗体与感觉性共济失调等感觉障碍为主的疾病相关•GQ1b与Miller-Fisher综合征（MFS),Bickerstaff脑干脑炎（BEE）等存在眼肌麻痹或球部肌肉受累的疾病相关Afterexogen

ousgangliosidesinjection•Hightitersofanti-GM1antibodieswerefoundinpatientswhodevelopedGBSfollowingexogenousgangliosidesinjection,leadingtothesuspicio

nthatexogenousgangliosidesmightbeforeigntohumansandmayactasanimmunogenicagent.•GBSfollowingintravenousu

seofgangliosidesinEuropeseveraldecadesago•ledtoitswithdrawalfromEuropeanmarketFiguerasA,Morales-OlivasFJ,CapellaD,Pa

lopV,LaporteJR(1992)Bovinegangliosidesandacutemotorpolyneuropathy.BMJ305:1330–1331.Weidentified7patientswhodevelopedGBSafterintravenoususe

ofgangliosides•DepartmentofNeurologyoftheFirstHospitalofJilinUniversity•2013•Gangliosidesasanexclusivecomponentorpartofacompoundhaveneverbee

nusedinourdepartmentandalltheenrolledpatientswerereferredtoourdepartmentfromotherdepartmentsorfromotherhospitals.Grouping•Enro

lledsubjectsweredividedintotheganglioside+group(ganglioside-associated)andtheganglioside-group(non-ganglioside-associated)accordingtowhethertheyre

ceivedexogenousgangliosidesbeforediseaseonset.Evaluationofclinicalseverityandfunctionalimpairment•MotorfunctiondeficitsofpatientswerescoredbytheHughe

sFunctionalGradingScale(HFGS)score•NeurologicfunctionwasalsoevaluatedbyusingtheMedicalResearchCouncil(MRC)sumscoreofsixbilat

eralmusclesinarmsandlegs,rangingfrom0(tetraparalytic)to60(normalstrength)TheassociationbetweenexogenousgangliosidesandGBS•highti

tersofanti-GM1antibodieswerefoundinsomeofthepatientswhodevelopedGBSafterreceivingagangliosidetherapy,leadingtothesuspicionthatexogenousgangliosid

esmightbeforeigntohumansandmightbeneuritogenicinhumans•TheassociationbetweenexogenousgangliosidesandGBSwasfurtherevidencedbythepositivityofanti

-GM1andanti-GT1aantibodiesinCSFandplasmaSum•Insum,exogenousgangliosidesmaybeassociatedwithdevelopmentofGBSduetoinc

ompletelyrecognizedpathogenesis.Ganglioside-associatedGBSismoresevereinclinicalcoursewithpoorershort-termprognosisascomparedwith

non-gangliosideassociatedGBSinnortheastChina.