急性炎癥性脫髓鞘性多發(fā)性神經(jīng)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(jīng)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(guān)。近來發(fā)現(xiàn)AMAN與GD1a抗體相關(guān)密切。臨床表現(xiàn)：中國病理：AMAN：IgG和補(bǔ)體在軸索周圍沉積，巨噬細(xì)胞侵入軸索周圍間隙，嚴(yán)重者有軸索變性。AIDP：IgG和補(bǔ)體在髓鞘外沉積，巨噬細(xì)胞也在髓鞘外，“撕開”髓鞘。AMSAN：感覺軸索比運(yùn)動(dòng)軸索損害重。EMG不能預(yù)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(guān)的自身免疫性疾病,

病原體某些成分與周圍神經(jīng)的髓鞘成分相似

Pathophysiology主要病理特點(diǎn)(principalcharacteristicofpathology)節(jié)段性脫髓鞘(segmentaldemyelization)小血管周圍炎性細(xì)胞浸潤C(jī)linicalmanifestations多數(shù)患者有前驅(qū)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(xì)胞分離AssessmentNerveconductionvelocitytestNerveconductionstudiesareadependableandearlydiagnosticindicatorofGBS.showsdemyelizationanddamagetothenervesheathF反應(yīng)、H反射異常PL延長，NCV減慢傳導(dǎo)阻滯現(xiàn)象，伴或不伴有波幅降低Assessment腓腸神經(jīng)活檢節(jié)段性脫髓鞘小血管周圍炎性細(xì)胞浸潤Electrocardiogram(EKG)Mayshowabnormalitiesincardiacrhythm心律失常SubtypesofGBS經(jīng)典型AIDPFisher綜合癥(MillerFishersyndrome)：三聯(lián)征-“眼外肌麻痹、共濟(jì)失調(diào)、腱反射消失”，還有中樞神經(jīng)系統(tǒng)損害

ItwasthoughttobeavariantofGBSandcomprisecompleteophthalmoplegiawithataxiaandareflexia腦神經(jīng)型SubtypesofGBS軸突型純運(yùn)動(dòng)型（AMAN）運(yùn)動(dòng)感覺型（AMSAN）急性感覺性多發(fā)性神經(jīng)炎（ASP）急性全自主神經(jīng)?。ˋPN）假性肌營養(yǎng)不良復(fù)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(jīng)損害，腦脊液正常電解質(zhì)（血鉀<3.5)及心電圖檢查異常補(bǔ)鉀治療有效Differentialdiagnosis重癥肌無力(myastheniagravis)骨骼肌病態(tài)易疲勞性、波動(dòng)性nosensorysymptomstendonreflexesareunimpaired

Differentialdiagnosis脊髓灰質(zhì)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(shí)氣管插管，使用呼吸器預(yù)防呼吸道感染翻身、拍背、稀化痰液、吸痰Generaltreatment預(yù)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(yù)防并發(fā)癥(preventionofcomplication)合理的正壓通氣、吸出分泌物經(jīng)常翻身，保持床單平整皮下應(yīng)用肝素有臨床指征時(shí)，應(yīng)用廣譜抗生素等Generaltreatment對(duì)癥處理必要時(shí)心電監(jiān)護(hù)高血壓—小劑量β受體阻滯劑低血壓—補(bǔ)液心動(dòng)過速—通常不需要治療心動(dòng)過緩—阿托品疼痛—卡馬西平Immunotherapy機(jī)理抑制免疫反應(yīng)，去除致病因子對(duì)神經(jīng)損害，使髓鞘有時(shí)間再生方法血漿置換靜脈注射免疫球蛋白皮質(zhì)醇激素治療PlasmaexchangeTheusefulnessofplasmaexchangeintheevolvingphaseofGBS.Inpatientswhoaretreatedwithin2weeksofonset,thereisareductionintheperiodofhospitalizationinthelengthoftimethatthepatientrequiresmechanicalventilation.However,whenplasmaexchangeisdelayedfor2weeksorlongeraftertheonsetofthedisease,theprocedurehas,withafewnotableexceptions,beenoflittlevalue.Plasmaexchange血漿置換機(jī)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(shè)備，價(jià)格昂貴適用于急性進(jìn)行性加重的GBS用法：40ml/kg禁忌癥：嚴(yán)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