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1、急性炎症性脱髓鞘性多发性神经病急性炎症性脱髓鞘性多发性神经病Acute Inflammatory Demyelinating Polyneuropathy, AIDPAcute Inflammatory Demyelinating Polyneuropathy, AIDP1多发性神经病 Introductionn nLandry Landry - -Landrys paralysis 1859Landrys paralysis 1859n nLandryreportedLandryreportedan acute, ascendingan acute, ascending, ,predomin
2、antlypredominantlymotorparalysiswithrespiratoryfailure,leadingtodeathmotorparalysiswithrespiratoryfailure,leadingtodeathn nGuillair-Barre19162Guillair-Barre19162例例 n nGuillain,Guillain, BarreBarre andand strohlstrohl (1916)(1916) reportedreported a a benignbenignpolyneuritispolyneuritis withwith alb
3、uminocytologic albuminocytologic dissociation dissociation in in the the CSF CSF (raised (raised concentration concentration of of CSF CSF protein protein but but a a normal normal cell count)cell count) 蛋白细胞分离蛋白细胞分离蛋白细胞分离蛋白细胞分离是本病的特征是本病的特征2多发性神经病GuillainBarre LandryStrohl3多发性神经病Introductionn nIn195
4、6,CMillerFisherdescribedatriadofacuteIn1956,CMillerFisherdescribedatriadofacuteophthalmoplegia,ataxia,andareflexia,nowknownasophthalmoplegia,ataxia,andareflexia,nowknownasFisherssyndromeFisherssyndromen nDuringthepast15years,GBShasbecomeclearthatDuringthepast15years,GBShasbecomeclearthatthisclinical
5、picture,nowcalledGuillain-Barrthisclinicalpicture,nowcalledGuillain-Barrsyndrome,andhavedifferentpathologicalsubtypessyndrome,andhavedifferentpathologicalsubtypes4多发性神经病Epidemiologyn nWorldwide incidencen n0.6-4/100000peryearthroughouttheworld0.6-4/100000peryearthroughouttheworldn nChina incidencen
6、n0.66per100000forallages0.66per100000forallagesn n可发生于任何年龄，男女发病率相似，夏秋多见可发生于任何年龄，男女发病率相似，夏秋多见可发生于任何年龄，男女发病率相似，夏秋多见可发生于任何年龄，男女发病率相似，夏秋多见5多发性神经病6多发性神经病临床表现：中国临床表现：中国n n儿童和青少年，夏初。n nEMG：轴索损害，AMAN。n nEMGEMG符合符合AMANAMAN的为的为6565，符合，符合AIDPAIDP的为的为2424。n n66有CJ抗体，42有GM1抗体，其他神经节苷脂抗体为1726。与西方国家不同，GM1抗体与AMAN或AI
7、DP无关。近来发现AMAN与GD1a抗体相关密切。7多发性神经病临床表现：中国临床表现：中国n n病理：n nAMANAMAN：IgGIgG和补体在轴索周围沉积，巨噬细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。胞侵入轴索周围间隙，严重者有轴索变性。n nAIDPAIDP：IgGIgG和补体在髓鞘外沉积，巨噬细胞也和补体在髓鞘外沉积，巨噬细胞也在髓鞘外，在髓鞘外，“ “撕开撕开” ”髓鞘。髓鞘。n nAMSANAMSAN：感觉轴索比运动轴索损害重。：感觉轴索比运动轴索损害重。n nEMG不能预测病理。8多发性神经病Pathogenesis and Pathophysiol
8、ogyn nThecauseofthissyndromeisunknown,butitisgenerallyviewedtobeanautoimmuneresponsetoabacterialorviralinfection.n n病因尚未完全阐明病因尚未完全阐明病因尚未完全阐明病因尚未完全阐明9多发性神经病Etiologyn nC CampylobacterampylobacterJ Jejuniejunin nEpstein-BarrVirusEpstein-BarrVirus(EBV)(EBV)n nCytomegalovirusCytomegalovirus(CMV)(CMV)n nH
9、IVHIVn nVaccinationsn n空肠肠弯曲菌空肠肠弯曲菌10多发性神经病Pathogenesis and Pathophysiologyn nAn acute immune-mediated polyneuropathy , An acute immune-mediated polyneuropathy , component of pathogen was similar with myelin component of pathogen was similar with myelin sheath of peripheral nervesheath of peripheral
10、 nerven n与感染有关的自身免疫性疾病与感染有关的自身免疫性疾病与感染有关的自身免疫性疾病与感染有关的自身免疫性疾病, , 病原体某些成分与周病原体某些成分与周病原体某些成分与周病原体某些成分与周围神经的髓鞘成分相似围神经的髓鞘成分相似围神经的髓鞘成分相似围神经的髓鞘成分相似 11多发性神经病Pathophysiologyn n主要病理特点主要病理特点主要病理特点主要病理特点(principal characteristic of (principal characteristic of pathology )pathology )n n节段性脱髓鞘节段性脱髓鞘节段性脱髓鞘节段性脱髓鞘(
11、segmental demyelization)(segmental demyelization)n n小血管周围炎性细胞浸润小血管周围炎性细胞浸润小血管周围炎性细胞浸润小血管周围炎性细胞浸润12多发性神经病13多发性神经病14多发性神经病15多发性神经病Clinical manifestationsn n多数患者有前驱症状多数患者有前驱症状多数患者有前驱症状多数患者有前驱症状( (起病前起病前起病前起病前1313周）周）周）周）n n呼吸道感染症状呼吸道感染症状呼吸道感染症状呼吸道感染症状n n喉痛、鼻塞、发热喉痛、鼻塞、发热喉痛、鼻塞、发热喉痛、鼻塞、发热n n消化道症状消化道症状消化道症
12、状消化道症状n n腹泻、呕吐腹泻、呕吐腹泻、呕吐腹泻、呕吐16多发性神经病Clinical manifestationsn nProgressiveascendingsymmetricalweaknessoftheProgressiveascendingsymmetricalweaknessofthelimbslimbsn nInvolvementofproximalanddistalmusclesInvolvementofproximalanddistalmusclesn nNumbnessandtinglinginthehandsandfeetNumbnessandtinglinginth
13、ehandsandfeetn nBackpainBackpain17多发性神经病Clinical manifestationsn nDepressedorabsentreflexesDepressedorabsentreflexesn nInvolvementofcranialnerves(facialnervesmostInvolvementofcranialnerves(facialnervesmostcommonlyinvolved)commonlyinvolved)n nRespiratoryfailure(involvedrespiratorymuscles)Respiratoryf
14、ailure(involvedrespiratorymuscles)n nProgressiontopeakdisabilityin4wkProgressiontopeakdisabilityin4wkn nautonomicnervesymptomautonomicnervesymptom18多发性神经病Assessmentn nCerebrospinalfluidn nIncreasedproteinusuallyafter7to10days.Increasedproteinusuallyafter7to10days.n nWhilesomeproteinisnormallypresent
15、,anWhilesomeproteinisnormallypresent,anincreasedamountwithoutanincreaseintheincreasedamountwithoutanincreaseinthenumberofwhitebloodcellsmayindicateGBSnumberofwhitebloodcellsmayindicateGBSn n蛋白细胞分离蛋白细胞分离19多发性神经病Assessmentn nNerveconductionvelocitytestn nNerveconductionstudiesareadependableandNervecon
16、ductionstudiesareadependableandearlydiagnosticindicatorofGBS.earlydiagnosticindicatorofGBS.n nshowsdemyelizationanddamagetothenerveshowsdemyelizationanddamagetothenervesheathsheathn nF F反应、反应、H H反射异常反射异常PLPL延长，延长，NCVNCV减慢减慢n n 传导阻滞现象，伴或不伴有波幅降低传导阻滞现象，伴或不伴有波幅降低20多发性神经病Assessmentn n腓肠神经活检腓肠神经活检n n节段性脱髓
17、鞘节段性脱髓鞘节段性脱髓鞘节段性脱髓鞘n n小血管周围炎性细胞浸润小血管周围炎性细胞浸润小血管周围炎性细胞浸润小血管周围炎性细胞浸润n nElectrocardiogram(EKG)n nMayshowabnormalitiesincardiacrhythmn n心律失常21多发性神经病Subtypes of GBS n n经典型经典型经典型经典型 AIDPAIDPn nFisherFisher综合症综合症综合症综合症(Miller Fisher syndrome )(Miller Fisher syndrome )：n n三联征三联征三联征三联征- -“ “眼外肌麻痹眼外肌麻痹眼外肌麻痹眼外
18、肌麻痹、 共济失调、腱反射消失共济失调、腱反射消失共济失调、腱反射消失共济失调、腱反射消失” ”，还还还还有中枢神经系统损害有中枢神经系统损害有中枢神经系统损害有中枢神经系统损害 n nItwasthoughttobeavariantofGBSandcompriseItwasthoughttobeavariantofGBSandcomprisecompleteophthalmoplegiawithataxiaandareflexiacompleteophthalmoplegiawithataxiaandareflexian n脑神经型脑神经型脑神经型脑神经型22多发性神经病Subtypes o
19、f GBS n n轴突型轴突型轴突型轴突型 n n纯运动型（纯运动型（纯运动型（纯运动型（AMANAMAN）n n运动运动运动运动 感觉感觉感觉感觉 型型型型 （AMSAN AMSAN ）n n急性感觉性多发性神经炎（急性感觉性多发性神经炎（急性感觉性多发性神经炎（急性感觉性多发性神经炎（ASPASP）n n急性全自主神经病（急性全自主神经病（急性全自主神经病（急性全自主神经病（APNAPN）n n假性肌营养不良假性肌营养不良假性肌营养不良假性肌营养不良n n复发型复发型复发型复发型23多发性神经病Diagnosisn nRequiredfordiagnosisn nProgressivewe
20、aknessofoneormorelimbProgressiveweaknessofoneormorelimbn nDistalareflexiawithproximalareflexiaorDistalareflexiawithproximalareflexiaorhyporeflexiahyporeflexia24多发性神经病Diagnosisn nSupportivediagnosisn nProgressionofsymptomsoverdaysto4wkProgressionofsymptomsoverdaysto4wkn nRelativesymmetryofdeficitsRel
21、ativesymmetryofdeficitsn nMildsensoryinvolvementMildsensoryinvolvementn nCranialnerveinvolvement(especiallyVII)Cranialnerveinvolvement(especiallyVII)n nRecoverybeginningwithin4wkRecoverybeginningwithin4wk25多发性神经病Diagnosisn nSupportivediagnosisn nAutonomicdysfunctionAutonomicdysfunctionn nNofeverNofe
22、vern nIncreasedCSFproteinafter1wkIncreasedCSFproteinafter1wkn nCSFwhitebloodcellcount10/LCSFwhitebloodcellcount10/Ln nNerveconductionslowingorblockedbyseveralNerveconductionslowingorblockedbyseveralweeksweeks26多发性神经病Diagnosisn nAgainstdiagnosisn nSignificantasymmetricweaknessSignificantasymmetricwea
23、knessn nBowelorbladderdysfunctionatonsetorpersistentBowelorbladderdysfunctionatonsetorpersistentn nCSFwhitebloodcellcount50orPMNcount0LCSFwhitebloodcellcount50orPMNcount0Ln nWell-demarcatedsensorylevelWell-demarcatedsensorylevel27多发性神经病Diagnosisn nExcludingdiagnosisn nIsolatedsensoryinvolvement,with
24、outweaknessIsolatedsensoryinvolvement,withoutweaknessn nAnotherpolyneuropathythatexplainsclinicalAnotherpolyneuropathythatexplainsclinicalpicturepicture28多发性神经病Differential diagnosisn nAcquiredhypokalemiaAcquiredhypokalemian nBotulismBotulismn nMyastheniagravisMyastheniagravisn nPeriodicparalysisPer
25、iodicparalysisn nPoliomyelitisPoliomyelitisn nPolymyositisPolymyositisn nTickparalysisTickparalysisn nDiphtheriaDiphtherian nTransversemyelitisTransversemyelitisn nHeavymetal(leadandHeavymetal(leadandarsenicpoisoning)arsenicpoisoning)29多发性神经病Differential diagnosisn n低钾性周期性瘫痪低钾性周期性瘫痪(hypokalemic peri
26、odic paralysis)n n无病前感染史，常有发作史无病前感染史，常有发作史无病前感染史，常有发作史无病前感染史，常有发作史n n无感觉和脑神经损害，脑脊液正常无感觉和脑神经损害，脑脊液正常无感觉和脑神经损害，脑脊液正常无感觉和脑神经损害，脑脊液正常n n电解质（血钾电解质（血钾电解质（血钾电解质（血钾3.5)3.5)及心电图检查异常及心电图检查异常及心电图检查异常及心电图检查异常n n补钾治疗有效补钾治疗有效补钾治疗有效补钾治疗有效30多发性神经病Differential diagnosisn n重症肌无力重症肌无力(myasthenia gravis)n n骨骼肌骨骼肌骨骼肌骨骼肌
27、 病病病病态态易疲易疲易疲易疲劳劳性、波性、波性、波性、波动动性性性性n nno sensory symptoms no sensory symptoms n ntendon reflexes are unimpairedtendon reflexes are unimpaired 31多发性神经病Differential diagnosisn n脊髓灰质炎脊髓灰质炎(poliomyelitis)n n早期出现括约肌功能障碍早期出现括约肌功能障碍早期出现括约肌功能障碍早期出现括约肌功能障碍n n无感觉障碍无感觉障碍无感觉障碍无感觉障碍n nFever,meningealsymptoms,ear
28、lypleocytosis, and purely motor and usuallyasymmetricalareflexicparalysis.32多发性神经病Differential diagnosisn n急性脊髓炎（acutemyelitis）n nTheimmediateproblemistodifferentiateGBSTheimmediateproblemistodifferentiateGBSfromacutespinalcorddisease(fromacutespinalcorddisease(markedbymarkedbysensorimotorparalysisb
29、elowalevelonthetrunksensorimotorparalysisbelowalevelonthetrunkandsphinctericparalysis).andsphinctericparalysis).33多发性神经病Clinical managementn nGeneral treatment 一般治疗一般治疗n nImmunotherapy 免疫治疗免疫治疗34多发性神经病General treatmentn n保持呼吸道通畅保持呼吸道通畅保持呼吸道通畅保持呼吸道通畅n n辅助呼吸辅助呼吸n n密切观察，测肺活量密切观察，测肺活量密切观察，测肺活量密切观察，测肺活量2
30、0ml/kgICU20ml/kgICU20ml/kgICU20ml/kgICU必要时气管必要时气管必要时气管必要时气管插管，使用呼吸器插管，使用呼吸器插管，使用呼吸器插管，使用呼吸器n n预防呼吸道感染预防呼吸道感染预防呼吸道感染预防呼吸道感染n n翻身、拍背、稀化痰液、吸痰翻身、拍背、稀化痰液、吸痰翻身、拍背、稀化痰液、吸痰翻身、拍背、稀化痰液、吸痰35多发性神经病36多发性神经病General treatmentn n预防并发症预防并发症(preventionofcomplication)(preventionofcomplication)n n坠积性肺炎坠积性肺炎坠积性肺炎坠积性肺炎n
31、n褥疮褥疮褥疮褥疮n n血栓性静脉炎血栓性静脉炎血栓性静脉炎血栓性静脉炎n n防止肢体挛缩防止肢体挛缩防止肢体挛缩防止肢体挛缩n n尿路感染尿路感染尿路感染尿路感染37多发性神经病General treatmentn n预防并发症预防并发症(preventionofcomplication)(preventionofcomplication)n n合理的正压通气、吸出分泌物合理的正压通气、吸出分泌物合理的正压通气、吸出分泌物合理的正压通气、吸出分泌物n n经常翻身，保持床单平整经常翻身，保持床单平整经常翻身，保持床单平整经常翻身，保持床单平整n n皮下应用肝素皮下应用肝素皮下应用肝素皮下应用肝
32、素n n有临床指征时，应用广谱抗生素等有临床指征时，应用广谱抗生素等有临床指征时，应用广谱抗生素等有临床指征时，应用广谱抗生素等38多发性神经病General treatmentn n对症处理对症处理n n必要时心电监护必要时心电监护必要时心电监护必要时心电监护n n高血压高血压高血压高血压小剂量小剂量小剂量小剂量受体阻滞剂受体阻滞剂受体阻滞剂受体阻滞剂n n低血压低血压低血压低血压补液补液补液补液n n心动过速心动过速心动过速心动过速通常不需要治疗通常不需要治疗通常不需要治疗通常不需要治疗n n心动过缓心动过缓心动过缓心动过缓阿托品阿托品阿托品阿托品n n疼痛疼痛疼痛疼痛卡马西平卡马西平卡马
33、西平卡马西平39多发性神经病Immunotherapyn n机理机理n n抑制免疫反应，去除致病因子对神经损害，使髓鞘有抑制免疫反应，去除致病因子对神经损害，使髓鞘有时间再生时间再生n n方法方法n n血浆置换血浆置换n n静脉注射免疫球蛋白静脉注射免疫球蛋白静脉注射免疫球蛋白静脉注射免疫球蛋白n n皮质醇激素治疗皮质醇激素治疗皮质醇激素治疗皮质醇激素治疗40多发性神经病Plasma exchangen nTheTheusefulnessusefulnessofofplasmaplasmaexchangeexchangeininthetheevolvingevolvingphasephaseo
34、fofGBSGBS. .n nInInpatientspatientswhowhoarearetreatedtreatedwithinwithin2 2weeksweeksofofonsetonset, ,therethereisisa areductionreductionininthetheperiodperiodofofhospitalizationhospitalizationininthethelengthlengthofoftimetimethatthepatientrequiresmechanicalventilation.thatthepatientrequiresmechan
35、icalventilation.n nHowever,However, whenwhen plasmaplasma exchangeexchange isis delayeddelayed forfor 2 2 weeksweeks ororlongerlongerafterafterthetheonsetonsetofofthethediseasedisease, ,thetheprocedureprocedurehas,has, withwitha afewnotableexceptions,beenfewnotableexceptions,beenoflittlevalue.oflitt
36、levalue.41多发性神经病Plasma exchangen n血浆置换血浆置换血浆置换血浆置换n n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，好，好，好，n n专用设备，价格昂贵专用设备，价格昂贵专用设备，价格昂贵专用设备，价格昂贵n n适用于急性进行性加重的适用于急性进行性加重的适用于急性进行性加重的适用于急性进行性加重的GBSGBSn n用法：用法：用法：用法：40ml/kg40ml/kgn
37、n禁忌症：严重感染，禁忌症：严重感染，禁忌症：严重感染，禁忌症：严重感染， 心律失常、心功能不全，心律失常、心功能不全，心律失常、心功能不全，心律失常、心功能不全， 凝血功能障碍凝血功能障碍凝血功能障碍凝血功能障碍42多发性神经病Intravenous immunoglobulinn n静脉注射免疫球蛋白静脉注射免疫球蛋白n n尽早施行尽早施行尽早施行尽早施行n n用法：用法：用法：用法：0.4g/(kg.d)50.4g/(kg.d)5天天天天n n禁忌症：免疫球蛋白过敏，先天性禁忌症：免疫球蛋白过敏，先天性禁忌症：免疫球蛋白过敏，先天性禁忌症：免疫球蛋白过敏，先天性IgAIgA缺乏缺乏缺乏缺
38、乏n n PE PE 和和和和IVIGIVIG不必联合应用不必联合应用不必联合应用不必联合应用43多发性神经病Corticosteroidsn n皮质类固醇皮质类固醇n n有争议有争议有争议有争议n n理论上合理理论上合理理论上合理理论上合理n n研究表明无效研究表明无效研究表明无效研究表明无效n n经验：青年人大剂量早期使用经验：青年人大剂量早期使用经验：青年人大剂量早期使用经验：青年人大剂量早期使用44多发性神经病Corticosteroidsn nTheThevaluevalueofofcorticosteroidscorticosteroidsininthethetreatmenttr
39、eatmentofofGBSGBShashasbeenbeendisputedfordecades.disputedfordecades.n nAlthoughAlthough corticosteroidscorticosteroids cancan nono longerlonger recommendedrecommended asasroutinetreatmentforacuteGBS.routinetreatmentforacuteGBS.n nWeWe havehave observedobserved a a fewfew instancesinstances inin whi
40、chwhich thethe intravenousintravenousadministrationadministration withwith high-closehigh-close corticosteroidscorticosteroids seeminglyseeminglyhaltedtheprogressofthedisease.haltedtheprogressofthedisease.45多发性神经病Prognosisn nPrognosisn nThemajorityofpatientsrecovercompletelyorThemajorityofpatientsrecovercompletelyornearlycompletelynearlycompletelyn nInabout10percentofpatients,theresidualInabout10percentofpatients,theresidualdisabilityispronounceddisabilityispronounced46多发性神经病47多发性神经病预后预后n n80%患者恢复完全n n死亡率大约5%(呼吸肌麻痹)48多发性神经病谢谢谢谢!49多发性神经病
