急性炎症性脱髓鞘性多发性神经病

GuillainBarreLandryStrohlIntroductionIn1956,CMillerFisherdescribedatriadofacuteophthalmoplegia,ataxia,andareflexia,nowknownasFisher’ssyndromeDuringthepast15years,GBShasbecomeclearthatthisclinicalpicture,nowcalledGuillain-Barrésyndrome,andhavedifferentpathologicalsubtypesEpidemiologyWorldwideincidence0.6-4/100000peryearthroughouttheworldChinaincidence0.66per100000forallages可发生于任何年龄，男女发病率相似，夏秋多见临床表现：中国儿童和青少年，夏初。EMG：轴索损害，AMAN。EMG符合AMAN的为65％，符合AIDP的为24％。66％有CJ抗体，42％有GM1抗体，其他神经节苷脂抗体为17－26％。与西方国家不同，GM1抗体与AMAN或AIDP无关。近来发现AMAN与GD1a抗体相关密切。临床表现：中国病理：AMAN：IgG和补体在轴索周围沉积，巨噬细胞侵入轴索周围间隙，严重者有轴索变性。AIDP：IgG和补体在髓鞘外沉积，巨噬细胞也在髓鞘外，“撕开”髓鞘。AMSAN：感觉轴索比运动轴索损害重。EMG不能预测病理。PathogenesisandPathophysiologyThecauseofthissyndromeisunknown,butitisgenerallyviewedtobeanautoimmuneresponsetoabacterialorviralinfection.病因尚未完全阐明EtiologyCampylobacterJejuniEpstein-BarrVirus(EBV)

Cytomegalovirus(CMV)HIVVaccinations···········空肠肠弯曲菌PathogenesisandPathophysiologyAnacuteimmune-mediatedpolyneuropathy,componentofpathogenwassimilarwithmyelinsheathofperipheralnerve与感染有关的自身免疫性疾病,

病原体某些成分与周围神经的髓鞘成分相似

Pathophysiology主要病理特点(principalcharacteristicofpathology)节段性脱髓鞘(segmentaldemyelization)小血管周围炎性细胞浸润Clinicalmanifestations多数患者有前驱症状(起病前1~3周）呼吸道感染症状喉痛、鼻塞、发热消化道症状腹泻、呕吐ClinicalmanifestationsProgressiveascendingsymmetricalweaknessofthelimbsInvolvementofproximalanddistalmusclesNumbnessandtinglinginthehandsandfeetBackpainClinicalmanifestationsDepressedorabsentreflexesInvolvementofcranialnerves(facialnervesmostcommonlyinvolved)Respiratoryfailure(involvedrespiratorymuscles)Progressiontopeakdisabilityin4wkautonomicnervesymptomAssessmentCerebrospinalfluidIncreasedproteinusuallyafter7to10days.Whilesomeproteinisnormallypresent,anincreasedamountwithoutanincreaseinthenumberofwhitebloodcellsmayindicateGBS蛋白细胞分离AssessmentNerveconductionvelocitytestNerveconductionstudiesareadependableandearlydiagnosticindicatorofGBS.showsdemyelizationanddamagetothenervesheathF反应、H反射异常PL延长，NCV减慢传导阻滞现象，伴或不伴有波幅降低Assessment腓肠神经活检节段性脱髓鞘小血管周围炎性细胞浸润Electrocardiogram(EKG)Mayshowabnormalitiesincardiacrhythm心律失常SubtypesofGBS经典型AIDPFisher综合症(MillerFishersyndrome)：三联征-“眼外肌麻痹、共济失调、腱反射消失”，还有中枢神经系统损害

ItwasthoughttobeavariantofGBSandcomprisecompleteophthalmoplegiawithataxiaandareflexia脑神经型SubtypesofGBS轴突型纯运动型（AMAN）运动感觉型（AMSAN）急性感觉性多发性神经炎（ASP）急性全自主神经病（APN）假性肌营养不良复发型DiagnosisRequiredfordiagnosisProgressiveweaknessofoneormorelimbDistalareflexiawithproximalareflexiaorhyporeflexiaDiagnosisSupportivediagnosisProgressionofsymptomsoverdaysto4wkRelativesymmetryofdeficitsMildsensoryinvolvementCranialnerveinvolvement(especiallyVII)Recoverybeginningwithin4wkDiagnosisSupportivediagnosisAutonomicdysfunctionNofeverIncreasedCSFproteinafter1wkCSFwhitebloodcellcount≤10/μLNerveconductionslowingorblockedbyseveralweeksDiagnosisAgainstdiagnosisSignificantasymmetricweaknessBowelorbladderdysfunctionatonsetorpersistentCSFwhitebloodcellcount>50orPMNcount>0μLWell-demarcatedsensorylevelDiagnosisExcludingdiagnosisIsolatedsensoryinvolvement,withoutweaknessAnotherpolyneuropathythatexplainsclinicalpictureDifferentialdiagnosisAcquiredhypokalemiaBotulismMyastheniagravisPeriodicparalysisPoliomyelitisPolymyositisTickparalysisDiphtheriaTransversemyelitisHeavymetal(leadandarsenicpoisoning)Differentialdiagnosis低钾性周期性瘫痪(hypokalemicperiodicparalysis)无病前感染史，常有发作史无感觉和脑神经损害，脑脊液正常电解质（血钾<3.5)及心电图检查异常补钾治疗有效Differentialdiagnosis重症肌无力(myastheniagravis)骨骼肌病态易疲劳性、波动性nosensorysymptomstendonreflexesareunimpaired

Differentialdiagnosis脊髓灰质炎(poliomyelitis)早期出现括约肌功能障碍无感觉障碍

Fever,meningealsymptoms,earlypleocytosis,andpurelymotorandusuallyasymmetricalareflexicparalysis.Differentialdiagnosis急性脊髓炎（acutemyelitis）TheimmediateproblemistodifferentiateGBSfromacutespinalcorddisease(markedbysensorimotorparalysisbelowalevelonthetrunkandsphinctericparalysis).ClinicalmanagementGeneraltreatment一般治疗Immunotherapy免疫治疗Generaltreatment保持呼吸道通畅辅助呼吸密切观察，测肺活量20ml/kg→ICU必要时气管插管，使用呼吸器预防呼吸道感染翻身、拍背、稀化痰液、吸痰Generaltreatment预防并发症(preventionofcomplication)坠积性肺炎褥疮血栓性静脉炎防止肢体挛缩尿路感染Generaltreatment预防并发症(preventionofcomplication)合理的正压通气、吸出分泌物经常翻身，保持床单平整皮下应用肝素有临床指征时，应用广谱抗生素等Generaltreatment对症处理必要时心电监护高血压—小剂量β受体阻滞剂低血压—补液心动过速—通常不需要治疗心动过缓—阿托品疼痛—卡马西平Immunotherapy机理抑制免疫反应，去除致病因子对神经损害，使髓鞘有时间再生方法血浆置换静脉注射免疫球蛋白皮质醇激素治疗PlasmaexchangeTheusefulnessofplasmaexchangeintheevolvingphaseofGBS.Inpatientswhoaretreatedwithin2weeksofonset,thereisareductionintheperiodofhospitalizationinthelengthoftimethatthepatientrequiresmechanicalventilation.However,whenplasmaexchangeisdelayedfor2weeksorlongeraftertheonsetofthedisease,theprocedurehas,withafewnotableexceptions,beenoflittlevalue.Plasmaexchange血浆置换机制：去除血浆中致病因子，可明显缩短病程，使用越早，疗效越好，专用设备，价格昂贵适用于急性进行性加重的GBS用法：40ml/kg禁忌症：严重感染，心律失常、心功能不全，凝血功能障碍Intravenousimmunoglobulin静脉注射免疫球蛋白尽早施行用法：0.4g/(kg.d)×5天禁忌症：免疫球蛋白过敏，先天性