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急性炎症性脱髓鞘性多发性神经病AcuteInflammatoryDemyelinatingPolyneuropathy,AIDP浙江大学医学院附属第一医院Introduction⚫Landry-Landry'sparalysis1859⚫Landryreportedanacute

,ascending,predominantlymotorparalysiswithrespiratoryfailure,leadingtodeath⚫Guillair-Barre19162例⚫Guillain,Barreandstrohl(1916)

reportedabenignpolyneuritiswithalbuminocytologicdissociationintheCSF(raisedconcentrationofCSFproteinbutanormalcellcount)蛋白细胞分离是本病的特征GuillainBarre

LandryStrohlIntroduction⚫In1956,CMillerFisherdescribedatriadofacuteophthalmoplegia,ataxia,andareflexia,n

owknownasFisher’ssyndrome⚫Duringthepast15years,GBShasbecomeclearthatthisclinicalpicture,nowcalledGuillai

n-Barrésyndrome,andhavedifferentpathologicalsubtypesEpidemiology⚫Worldwideincidence⚫0.6-4/100000peryearthroughouttheworld⚫Chinaincidence⚫0.66per1000

00forallages⚫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。PathogenesisandPathophysio

logy⚫Thecauseofthissyndromeisunknown,butitisgenerallyviewedtobeanautoimmuneresponsetoabacterialorviralinfection.⚫病因尚未完全阐明Etiology⚫Camp

ylobacterJejuni⚫Epstein-BarrVirus(EBV)⚫Cytomegalovirus(CMV)⚫HIV⚫Vaccinations⚫···········空肠肠弯曲菌PathogenesisandPathophysiology⚫Anacuteimmune-media

tedpolyneuropathy,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(princ

ipalcharacteristicofpathology)⚫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Clinicalmanifestations⚫Progressiveascendingsymmetricalweaknessofthelimbs⚫Involvementofproxim

alanddistalmuscles⚫Numbnessandtinglinginthehandsandfeet⚫BackpainClinicalmanifestations⚫Depressedorabsentreflexes⚫Involvem

entofcranialnerves(facialnervesmostcommonlyinvolved)⚫Respiratoryfailure(involvedrespiratorymuscles)⚫Progressiontopeakdisabilityin4wk⚫autonomicnerves

ymptomAssessment⚫Cerebrospinalfluid⚫Increasedproteinusuallyafter7to10days.⚫Whilesomeproteinisnormallypresent,ani

ncreasedamountwithoutanincreaseinthenumberofwhitebloodcellsmayindicateGBS⚫蛋白细胞分离Assessment⚫Nerveconductionveloc

itytest⚫NerveconductionstudiesareadependableandearlydiagnosticindicatorofGBS.⚫showsdemyelizationanddamagetothenerv

esheath⚫F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AIDP⚫Fisher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⚫Itwasthoughttobea

variantofGBSandcomprisecompleteophthalmoplegiawithataxiaandareflexia⚫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Diagnosis⚫Requiredfordiagnosis⚫Progressiveweaknessofoneormorelimb⚫Distalare

flexiawithproximalareflexiaorhyporeflexiaDiagnosis⚫Supportivediagnosis⚫Progressionofsymptomsoverdaysto4wk⚫Relativesymmetryofdeficits⚫Mildsensoryinvo

lvement⚫Cranialnerveinvolvement(especiallyVII)⚫Recoverybeginningwithin4wkDiagnosis⚫Supportivediagnosis⚫Autonomicd

ysfunction⚫Nofever⚫IncreasedCSFproteinafter1wk⚫CSFwhitebloodcellcount≤10/μL⚫Nerveconductionslowingorblock

edbyseveralweeksDiagnosis⚫Againstdiagnosis⚫Significantasymmetricweakness⚫Bowelorbladderdysfunctionatonsetorpersisten

t⚫CSFwhitebloodcellcount>50orPMNcount>0μL⚫Well-demarcatedsensorylevelDiagnosis⚫Excludingdiagnosis⚫Isolatedsensoryinvolvement,withoutweakness⚫Anot

herpolyneuropathythatexplainsclinicalpictureDifferentialdiagnosis⚫Acquiredhypokalemia⚫Botulism⚫Myastheniagravis⚫Periodicparalys

is⚫Poliomyelitis⚫Polymyositis⚫Tickparalysis⚫Diphtheria⚫Transversemyelitis⚫Heavymetal(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⚫nosensorysymptoms⚫tendonreflexesareunimpairedDifferentialdiagnosis⚫脊髓灰质炎(poliomyelitis)⚫早期出现括约肌功能障碍⚫无感觉障碍⚫Fever,meningealsymptoms,early

pleocytosis,andpurelymotorandusuallyasymmetricalareflexicparalysis.Differentialdiagnosis⚫急性脊髓炎（acutemyelitis）⚫Theimmediateproblemist

odifferentiateGBSfromacutespinalcorddisease(markedbysensorimotorparalysisbelowalevelonthetrunkandsphinctericparalysis).

Clinicalmanagement⚫Generaltreatment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Ge

neraltreatment⚫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Plasmaexchange⚫Theus

efulnessofplasmaexchangeintheevolvingphaseofGBS.⚫Inpatientswhoaretreatedwithin2weeksofonset,thereisareductionintheperiodofh

ospitalizationinthelengthoftimethatthepatientrequiresmechanicalventilation.⚫However,whenplasmaexchangeisdelayedfor2weeksor

longeraftertheonsetofthedisease,theprocedurehas,withafewnotableexceptions,beenoflittlevalue.Plasmaexchan

ge⚫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IgA缺乏⚫PE和IVIG不必联合应用Corticosteroids⚫皮质类固醇⚫有争议⚫理论上合理⚫研究表明无效⚫经验：青年人大剂量早期使用Corticosteroids⚫Theval

ueofcorticosteroidsinthetreatmentofGBShasbeendisputedfordecades.⚫AlthoughcorticosteroidscannolongerrecommendedasroutinetreatmentforacuteGBS.⚫Wehaveo

bservedafewinstancesinwhichtheintravenousadministrationwithhigh-closecorticosteroidsseeminglyhaltedt

heprogressofthedisease.Prognosis⚫Prognosis⚫Themajorityofpatientsrecovercompletelyornearlycompletely⚫Inabout10percentofpatients,theresidua

ldisabilityispronounced预后⚫80%患者恢复完全⚫死亡率大约5%(呼吸肌麻痹)