急性炎症性脱髓鞘性多发性神经病 .ppt

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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,AIDP浙江大学医学院附属第一医院浙江大学医学院附属第一医院 Introductionn nLandry Landry-Landrys paralysis 1859Landrys paralysis 1859n nLandryreportedLandryreportedan acute,ascendingan acute,ascendin

2、g,predominantlypredominantlymotorparalysiswithrespiratoryfailure,leadingtodeathmotorparalysiswithrespiratoryfailure,leadingtodeathn nGuillair-Barre 1916 2Guillair-Barre 1916 2例例 n nGuillain,Guillain,BarreBarre andand strohlstrohl (1916)(1916)reportedreported a a benignbenignpolyneuritispolyneuritis

3、withwith albuminocytologic 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GuillainBarre LandryStrohlIntroductionn nIn 1956,C

4、Miller Fisher described a triad of acute In 1956,C Miller Fisher described a triad of acute ophthalmoplegia,ataxia,and areflexia,now known as ophthalmoplegia,ataxia,and areflexia,now known as Fishers syndromeFishers syndromen nDuring the past 15 years,GBS has become clear that During the past 15 yea

5、rs,GBS has become clear that this clinical picture,now called Guillain-Barr this clinical picture,now called Guillain-Barr syndrome,and have different pathological subtypes syndrome,and have different pathological subtypes Epidemiologyn nWorldwide incidencen n0.6-4/100 000 per year throughout the wo

6、rld0.6-4/100 000 per year throughout the worldn nChina incidencen n0.66 per 100 000 for all ages0.66 per 100 000 for all agesn 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，夏秋多见临床表现：中国临床表现：中国n儿童和青少年，夏初。nEMG：轴索损害，AMAN。nEMG符合AMAN的为65，符合AIDP的为24。n66有CJ抗体，42有GM1抗体，其他神经

7、节苷脂抗体为1726。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：中国临床表现：中国n病理：nAMAN：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。nAIDP：IgG和补体在髓鞘外沉积，巨噬细胞也在髓鞘外，“撕开”髓鞘。nAMSAN：感觉轴索比运动轴索损害重。nEMG不能预测病理。Pathogenesis and Pathophysiologyn nThe cause of this syndrome is unknown,but it is generally viewed to be an autoimm

8、une response to a bacterial or viral infection.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Etiologyn nC CampylobacterampylobacterJ Jejuniejunin nEpstein-BarrVirusEpstein-BarrVirus(EBV)(EBV)n nCytomegalovirusCytomegalovirus(CMV)(CMV)n nHIVHIVn nVaccinationsn n空肠肠弯曲菌空肠肠弯曲菌Pathogenesis and Pathophysiologyn nAn

9、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 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,病原体某些成分与病原体某些成分与病原体某些成分

10、与病原体某些成分与周围神经的髓鞘成分相似周围神经的髓鞘成分相似周围神经的髓鞘成分相似周围神经的髓鞘成分相似 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(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Cl

11、inical 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状消化道症状n n腹泻、呕吐腹泻、呕吐腹泻、呕吐腹泻、呕吐Clinical manifestationsn nProgressive ascending symmetrical weakness of the Progressive ascending symme

12、trical weakness of the limbslimbsn nInvolvement of proximal and distal musclesInvolvement of proximal and distal musclesn nNumbness and tingling in the hands and feetNumbness and tingling in the hands and feetn nBack painBack painClinical manifestationsn nDepressed or absent reflexesDepressed or abs

13、ent reflexesn nInvolvement of cranial nerves(facial nerves most Involvement of cranial nerves(facial nerves most commonly involved)commonly involved)n nRespiratory failure(involved respiratory muscles)Respiratory failure(involved respiratory muscles)n nProgression to peak disability in 4 wkProgressi

14、on to peak disability in 4 wkn nautonomic nerve symptom autonomic nerve symptom Assessmentn nCerebrospinal fluidn nIncreased protein usually after 7 to 10 days.Increased protein usually after 7 to 10 days.n nWhile some protein is normally present,an increased While some protein is normally present,a

15、n increased amount without an increase in the number of white amount without an increase in the number of white blood cells may indicate GBSblood cells may indicate GBSn n蛋白细胞分离蛋白细胞分离Assessmentn nNerve conduction velocity testn n Nerve conduction studies are a dependable and Nerve conduction studies

16、 are a dependable and early diagnostic indicator of GBS.early diagnostic indicator of GBS.n nshows demyelization and damage to the nerve shows demyelization and damage to the nerve sheathsheathn 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Assessmentn n腓肠神经活检腓肠神经活检n

17、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 nMay show abnormalities in cardiac rhythmn n心律失常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肌麻痹

18、、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Subtypes of GBS n n轴突型轴突

19、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Diagnosisn nRequired for diagnosisn nProgressive weakness of one or mor

20、e limbProgressive weakness of one or more limbn nDistal areflexia with proximal areflexia or Distal areflexia with proximal areflexia or hyporeflexiahyporeflexiaDiagnosisn nSupportive diagnosisn nProgression of symptoms over days to 4 wkProgression of symptoms over days to 4 wkn nRelative symmetry o

21、f deficits Relative symmetry of deficits n nMild sensory involvementMild sensory involvementn nCranial nerve involvement(especially VII)Cranial nerve involvement(especially VII)n nRecovery beginning within 4 wkRecovery beginning within 4 wkDiagnosisn nSupportive diagnosisn nAutonomic dysfunctionAuto

22、nomic dysfunctionn nNo fever No fever n nIncreased CSF protein after 1 wkIncreased CSF protein after 1 wkn nCSF white blood cell count 10/LCSF white blood cell count 10/Ln nNerve conduction slowing or blocked by several Nerve conduction slowing or blocked by several weeksweeksDiagnosisn nAgainst dia

23、gnosisn nSignificant asymmetric weaknessSignificant asymmetric weaknessn nBowel or bladder dysfunction at onset or persistentBowel or bladder dysfunction at onset or persistentn nCSF white blood cell count 50 or PMN count 0LCSF white blood cell count 50 or PMN count 0Ln nWell-demarcated sensory leve

24、lWell-demarcated sensory levelDiagnosisn nExcluding diagnosisn nIsolated sensory involvement,without weaknessIsolated sensory involvement,without weaknessn nAnother polyneuropathy that explains clinical pictureAnother polyneuropathy that explains clinical pictureDifferential diagnosisn nAcquired hyp

25、okalemiaAcquired hypokalemian nBotulismBotulismn nMyasthenia gravisMyasthenia gravisn nPeriodic paralysisPeriodic paralysisn nPoliomyelitisPoliomyelitisn nPolymyositisPolymyositisn nTick paralysisTick paralysisn nDiphtheriaDiphtherian nTransverse myelitisTransverse myelitisn nHeavy metal(lead and He

26、avy metal(lead and arsenic poisoning)arsenic poisoning)Differential diagnosisn n低钾性周期性瘫痪低钾性周期性瘫痪(hypokalemic periodic 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

27、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 病病病病态态态态易疲易疲易疲易疲劳劳劳劳性、波性、波性、波性、波动动动动性性性性n nno sensory symptoms no sensory symptoms n ntendon reflexes are unimpairedtendon reflexes are unimpaired Differential diagnosisn n脊髓灰质炎脊髓灰质炎(poliomyelit

28、is)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,earlypleocytosis,and purely motor and usuallyasymmetricalareflexicparalysis.Differential diagnosisn n急性脊髓炎（acutemyelitis）n nTheimmediateproblemistodifferentiateGBSTheimmediateproblemistodifferentiateG

29、BSfromacutespinalcorddisease(fromacutespinalcorddisease(markedbymarkedbysensorimotorparalysisbelowalevelonthetrunksensorimotorparalysisbelowalevelonthetrunkandsphinctericparalysis).andsphinctericparalysis).Clinical managementn nGeneral treatment 一般治疗一般治疗n nImmunotherapy 免疫治疗免疫治疗General treatmentn n保

30、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20ml/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General treatmentn n预防并发症预防并发症(prevention of

31、complication)(prevention of complication)n n坠积性肺炎坠积性肺炎坠积性肺炎坠积性肺炎n 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General treatmentn n预防并发症预防并发症(prevention of complication)(prevention of complication)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

32、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

33、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西平卡马西平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Plasma exchangen nTheTheusefulnessusefulnessofofplasmaplasmaexchangeexchang

34、eininthetheevolvingevolvingphasephaseofofGBSGBS.n nInInpatientspatientswhowhoarearetreatedtreatedwithinwithin2 2weeksweeksofofonsetonset,therethereisisa areductionreductionininthetheperiodperiodofofhospitalizationhospitalizationininthethelengthlengthofoftimetimethatthepatientrequiresmechanicalventil

35、ation.thatthepatientrequiresmechanicalventilation.n nHowever,However,whenwhen plasmaplasma exchangeexchange isis delayeddelayed forfor 2 2 weeksweeks ororlongerlongerafterafterthetheonsetonsetofofthethediseasedisease,thetheprocedureprocedurehas,has,withwitha afewnotableexceptions,beenfewnotableexcep

36、tions,beenoflittlevalue.oflittlevalue.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：

37、用法：用法：40ml/kg40ml/kgn 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I

38、gAIgA缺乏缺乏缺乏缺乏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Corticosteroidsn nTheThevaluevalueofofcorticosteroidscorticosteroidsininthethetreatmenttreat

39、mentofofGBSGBShashasbeenbeendisputedfordecades.disputedfordecades.n nAlthoughAlthough corticosteroidscorticosteroids cancan nono longerlonger recommendedrecommended asasroutinetreatmentforacuteGBS.routinetreatmentforacuteGBS.n nWeWe havehaveobservedobserved a afewfew instancesinstancesininwhichwhich

40、thetheintravenousintravenousadministrationadministration withwith high-closehigh-close corticosteroidscorticosteroids seeminglyseeminglyhaltedtheprogressofthedisease.haltedtheprogressofthedisease.Prognosisn nPrognosisn nThe majority of patients recover completely or The majority of patients recover completely or nearly completely nearly completely n nIn about 10 percent of patients,the residual disability In about 10 percent of patients,the residual disability is pronouncedis pronounced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)