急性炎癥性脫髓鞘性多發(fā)性神經病

GuillainBarreLandryStrohlIntroductionIn1956,CMillerFisherdescribedatriadofacuteophthalmoplegia,ataxia,andareflexia,nowknownasFisher’ssyndromeDuringthepast15years,GBShasbecomeclearthatthisclinicalpicture,nowcalledGuillain-Barrésyndrome,andhavedifferentpathologicalsubtypesEpidemiologyWorldwideincidence0.6-4/100000peryearthroughouttheworldChinaincidence0.66per100000forallages可發(fā)生于任何年齡，男女發(fā)病率相似，夏秋多見臨床表現(xiàn)：中國兒童和青少年，夏初。EMG：軸索損害，AMAN。EMG符合AMAN的為65％，符合AIDP的為24％。66％有CJ抗體，42％有GM1抗體，其他神經節(jié)苷脂抗體為17－26％。與西方國家不同，GM1抗體與AMAN或AIDP無關。近來發(fā)現(xiàn)AMAN與GD1a抗體相關密切。臨床表現(xiàn)：中國病理：AMAN：IgG和補體在軸索周圍沉積，巨噬細胞侵入軸索周圍間隙，嚴重者有軸索變性。AIDP：IgG和補體在髓鞘外沉積，巨噬細胞也在髓鞘外，“撕開”髓鞘。AMSAN：感覺軸索比運動軸索損害重。EMG不能預測病理。PathogenesisandPathophysiologyThecauseofthissyndromeisunknown,butitisgenerallyviewedtobeanautoimmuneresponsetoabacterialorviralinfection.病因尚未完全闡明EtiologyCampylobacterJejuniEpstein-BarrVirus(EBV)

Cytomegalovirus(CMV)HIVVaccinations···········空腸腸彎曲菌PathogenesisandPathophysiologyAnacuteimmune-mediatedpolyneuropathy,componentofpathogenwassimilarwithmyelinsheathofperipheralnerve與感染有關的自身免疫性疾病,

病原體某些成分與周圍神經的髓鞘成分相似

Pathophysiology主要病理特點(principalcharacteristicofpathology)節(jié)段性脫髓鞘(segmentaldemyelization)小血管周圍炎性細胞浸潤Clinicalmanifestations多數(shù)患者有前驅癥狀(起病前1~3周）呼吸道感染癥狀喉痛、鼻塞、發(fā)熱消化道癥狀腹瀉、嘔吐ClinicalmanifestationsProgressiveascendingsymmetricalweaknessofthelimbsInvolvementofproximalanddistalmusclesNumbnessandtinglinginthehandsandfeetBackpainClinicalmanifestationsDepressedorabsentreflexesInvolvementofcranialnerves(facialnervesmostcommonlyinvolved)Respiratoryfailure(involvedrespiratorymuscles)Progressiontopeakdisabilityin4wkautonomicnervesymptomAssessmentCerebrospinalfluidIncreasedproteinusuallyafter7to10days.Whilesomeproteinisnormallypresent,anincreasedamountwithoutanincreaseinthenumberofwhitebloodcellsmayindicateGBS蛋白細胞分離AssessmentNerveconductionvelocitytestNerveconductionstudiesareadependableandearlydiagnosticindicatorofGBS.showsdemyelizationanddamagetothenervesheathF反應、H反射異常PL延長，NCV減慢傳導阻滯現(xiàn)象，伴或不伴有波幅降低Assessment腓腸神經活檢節(jié)段性脫髓鞘小血管周圍炎性細胞浸潤Electrocardiogram(EKG)Mayshowabnormalitiesincardiacrhythm心律失常SubtypesofGBS經典型AIDPFisher綜合癥(MillerFishersyndrome)：三聯(lián)征-“眼外肌麻痹、共濟失調、腱反射消失”，還有中樞神經系統(tǒng)損害

ItwasthoughttobeavariantofGBSandcomprisecompleteophthalmoplegiawithataxiaandareflexia腦神經型SubtypesofGBS軸突型純運動型（AMAN）運動感覺型（AMSAN）急性感覺性多發(fā)性神經炎（ASP）急性全自主神經?。ˋPN）假性肌營養(yǎng)不良復發(fā)型DiagnosisRequiredfordiagnosisProgressiveweaknessofoneormorelimbDistalareflexiawithproximalareflexiaorhyporeflexiaDiagnosisSupportivediagnosisProgressionofsymptomsoverdaysto4wkRelativesymmetryofdeficitsMildsensoryinvolvementCranialnerveinvolvement(especiallyVII)Recoverybeginningwithin4wkDiagnosisSupportivediagnosisAutonomicdysfunctionNofeverIncreasedCSFproteinafter1wkCSFwhitebloodcellcount≤10/μLNerveconductionslowingorblockedbyseveralweeksDiagnosisAgainstdiagnosisSignificantasymmetricweaknessBowelorbladderdysfunctionatonsetorpersistentCSFwhitebloodcellcount>50orPMNcount>0μLWell-demarcatedsensorylevelDiagnosisExcludingdiagnosisIsolatedsensoryinvolvement,withoutweaknessAnotherpolyneuropathythatexplainsclinicalpictureDifferentialdiagnosisAcquiredhypokalemiaBotulismMyastheniagravisPeriodicparalysisPoliomyelitisPolymyositisTickparalysisDiphtheriaTransversemyelitisHeavymetal(leadandarsenicpoisoning)Differentialdiagnosis低鉀性周期性癱瘓(hypokalemicperiodicparalysis)無病前感染史，常有發(fā)作史無感覺和腦神經損害，腦脊液正常電解質（血鉀<3.5)及心電圖檢查異常補鉀治療有效Differentialdiagnosis重癥肌無力(myastheniagravis)骨骼肌病態(tài)易疲勞性、波動性nosensorysymptomstendonreflexesareunimpaired

Differentialdiagnosis脊髓灰質炎(poliomyelitis)早期出現(xiàn)括約肌功能障礙無感覺障礙

Fever,meningealsymptoms,earlypleocytosis,andpurelymotorandusuallyasymmetricalareflexicparalysis.Differentialdiagnosis急性脊髓炎（acutemyelitis）TheimmediateproblemistodifferentiateGBSfromacutespinalcorddisease(markedbysensorimotorparalysisbelowalevelonthetrunkandsphinctericparalysis).ClinicalmanagementGeneraltreatment一般治療Immunotherapy免疫治療Generaltreatment保持呼吸道通暢輔助呼吸密切觀察，測肺活量20ml/kg→ICU必要時氣管插管，使用呼吸器預防呼吸道感染翻身、拍背、稀化痰液、吸痰Generaltreatment預防并發(fā)癥(preventionofcomplication)墜積性肺炎褥瘡血栓性靜脈炎防止肢體攣縮尿路感染Generaltreatment預防并發(fā)癥(preventionofcomplication)合理的正壓通氣、吸出分泌物經常翻身，保持床單平整皮下應用肝素有臨床指征時，應用廣譜抗生素等Generaltreatment對癥處理必要時心電監(jiān)護高血壓—小劑量β受體阻滯劑低血壓—補液心動過速—通常不需要治療心動過緩—阿托品疼痛—卡馬西平Immunotherapy機理抑制免疫反應，去除致病因子對神經損害，使髓鞘有時間再生方法血漿置換靜脈注射免疫球蛋白皮質醇激素治療PlasmaexchangeTheusefulnessofplasmaexchangeintheevolvingphaseofGBS.Inpatientswhoaretreatedwithin2weeksofonset,thereisareductionintheperiodofhospitalizationinthelengthoftimethatthepatientrequiresmechanicalventilation.However,whenplasmaexchangeisdelayedfor2weeksorlongeraftertheonsetofthedisease,theprocedurehas,withafewnotableexceptions,beenoflittlevalue.Plasmaexchange血漿置換機制：去除血漿中致病因子，可明顯縮短病程，使用越早，療效越好，專用設備，價格昂貴適用于急性進行性加重的GBS用法：40ml/kg禁忌癥：嚴重感染，心律失常、心功能不全，凝血功能障礙Intravenousimmunoglobulin靜脈注射免疫球蛋白盡早施行用法：0.4g/(kg.d)×5天禁忌癥：免疫球蛋白過敏，先天性