急性炎症性脱髓鞘性-多发性神经病ppt课件.ppt

急性炎症性脱髓鞘性多急性炎症性脱髓鞘性多发性神性神经病病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 1.Introductionn nLandry Landry-Landrys paralysis 1859Landrys paralysis 1859n nLandryreportedLandryreportedan acute,ascendingan acute,ascending,predominantlypredominantlymotorparalysiswithrespiratoryfailure,leadingtodeathmotorparalysiswithrespiratoryfailure,leadingtodeathn nGuillair-Barre 1916 2Guillair-Barre 1916 2例例 n nGuillain,Guillain,BarreBarre andand strohlstrohl (1916)(1916)reportedreported a a benignbenignpolyneuritispolyneuritis 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)蛋白蛋白蛋白蛋白细细胞分离胞分离胞分离胞分离是本病的特征是本病的特征2.GuillainBarre LandryStrohl3.Introductionn nIn 1956,C 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 years,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 4.Epidemiologyn nWorldwide incidencen n0.6-4/100 000 per year throughout the world0.6-4/100 000 per year throughout the worldn nChina incidencen n0.66 per 100 000 for all ages0.66 per 100 000 for all agesn n可可可可发发生于任何年生于任何年生于任何年生于任何年龄龄，男女，男女，男女，男女发发病率相似，夏秋多病率相似，夏秋多病率相似，夏秋多病率相似，夏秋多见见5.6.临临床表床表现现：中国：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的的为为6565，符合，符合AIDPAIDP的的为为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AIDP无关。近来发现AMAN与GD1a抗体相关密切。7.临临床表床表现现：中国：中国n n病理：n nAMANAMAN：IgGIgG和和补补体在体在轴轴索周索周围围沉沉积积，巨噬，巨噬细细胞胞侵入侵入轴轴索周索周围间围间隙，隙，严严重者有重者有轴轴索索变变性。性。n nAIDPAIDP：IgGIgG和和补补体在髓鞘外沉体在髓鞘外沉积积，巨噬，巨噬细细胞也胞也在髓鞘外，在髓鞘外，“撕开撕开”髓鞘。髓鞘。n nAMSANAMSAN：感：感觉轴觉轴索比运索比运动轴动轴索索损损害重。害重。n nEMG不能预测病理。8.Pathogenesis and Pathophysiologyn nThe cause of this syndrome is unknown,but it is generally viewed to be an autoimmune response to a bacterial or viral infection.n n病因尚未完全病因尚未完全病因尚未完全病因尚未完全阐阐明明明明9.Etiologyn nC CampylobacterampylobacterJ Jejuniejunin nEpstein-BarrVirusEpstein-BarrVirus(EBV)(EBV)n nCytomegalovirusCytomegalovirus(CMV)(CMV)n nHIVHIVn nVaccinationsn n空空空空肠肠肠肠弯曲菌弯曲菌弯曲菌弯曲菌10.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 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与病原体某些成分与周周周周围围神神神神经经的髓鞘成分相似的髓鞘成分相似的髓鞘成分相似的髓鞘成分相似 11.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节节段性脱髓鞘段性脱髓鞘段性脱髓鞘段性脱髓鞘(segmental demyelization)(segmental demyelization)n n小血管周小血管周小血管周小血管周围围炎性炎性炎性炎性细细胞浸胞浸胞浸胞浸润润12.13.14.15.Clinical manifestationsn n多数患者有前多数患者有前多数患者有前多数患者有前驱驱症状症状症状症状(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、喉痛、鼻塞、喉痛、鼻塞、喉痛、鼻塞、发热发热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16.Clinical manifestationsn nProgressive ascending symmetrical weakness of the Progressive ascending symmetrical 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 pain17.Clinical manifestationsn nDepressed or absent reflexesDepressed or absent 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 wkProgression to peak disability in 4 wkn nautonomic nerve symptom autonomic nerve symptom 18.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,an 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蛋白蛋白细细胞分离胞分离19.Assessmentn nNerve conduction velocity testn n Nerve conduction studies are a dependable and Nerve conduction studies 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反反应应、H H反射异常反射异常 PLPL延延长长，NCVNCV减慢减慢n n 传导传导阻滞阻滞现现象，伴或不伴有波幅降低象，伴或不伴有波幅降低20.Assessmentn n腓腓肠神神经活活检n n节节段性脱髓鞘段性脱髓鞘段性脱髓鞘段性脱髓鞘n n小血管周小血管周小血管周小血管周围围炎性炎性炎性炎性细细胞浸胞浸胞浸胞浸润润n nElectrocardiogram(EKG)n nMay show abnormalities in cardiac rhythmn n心律失常21.Subtypes of GBS n n经经典型典型典型典型 AIDPAIDPn nFisherFisher综综合症合症合症合症(Miller Fisher syndrome)(Miller Fisher syndrome)：n n三三三三联联征征征征-“眼外肌麻痹眼外肌麻痹眼外肌麻痹眼外肌麻痹、共共共共济济失失失失调调、腱反射消失、腱反射消失、腱反射消失、腱反射消失”，还还有中枢神有中枢神有中枢神有中枢神经经系系系系统损统损害害害害 n nItwasthoughttobeavariantofGBSandcompriseItwasthoughttobeavariantofGBSandcomprisecompleteophthalmoplegiawithataxiaandareflexiacompleteophthalmoplegiawithataxiaandareflexian n脑脑神神神神经经型型型型22.Subtypes of GBS n n轴轴突型突型突型突型 n n纯纯运运运运动动型（型（型（型（AMANAMAN）n n运运运运动动 感感感感觉觉 型型型型 （AMSAN AMSAN）n n急性感急性感急性感急性感觉觉性多性多性多性多发发性神性神性神性神经经炎（炎（炎（炎（ASPASP）n n急性全自主神急性全自主神急性全自主神急性全自主神经经病（病（病（病（APNAPN）n n假性肌假性肌假性肌假性肌营营养不良养不良养不良养不良n n复复复复发发型型型型23.Diagnosisn nRequired for diagnosisn nProgressive weakness of one or more limbProgressive weakness of one or more limbn nDistal areflexia with proximal areflexia or Distal areflexia with proximal areflexia or hyporeflexiahyporeflexia24.Diagnosisn nSupportive diagnosisn nProgression of symptoms over days to 4 wkProgression of symptoms over days to 4 wkn nRelative symmetry of 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 wk25.Diagnosisn nSupportive diagnosisn nAutonomic dysfunctionAutonomic 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 weeksweeks26.Diagnosisn nAgainst diagnosisn 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 levelWell-demarcated sensory level27.Diagnosisn nExcluding diagnosisn nIsolated sensory involvement,without weaknessIsolated sensory involvement,without weaknessn nAnother polyneuropathy that explains clinical pictureAnother polyneuropathy that explains clinical picture28.Differential diagnosisn nAcquired hypokalemiaAcquired hypokalemian nBotulismBotulismn nMyasthenia gravisMyasthenia gravisn nPeriodic paralysisPeriodic paralysisn nPoliomyelitisPoliomyelitisn nPolymyositisPolymyositisn nTick paralysisTick paralysisn nDiphtheriaDiphtherian nTransverse myelitisTransverse myelitisn nHeavy metal(lead and Heavy metal(lead and arsenic poisoning)arsenic poisoning)29.Differential diagnosisn n低低钾性周期性性周期性瘫痪(hypokalemic periodic paralysis)n n无病前感染史，常有无病前感染史，常有无病前感染史，常有无病前感染史，常有发发作史作史作史作史n n无感无感无感无感觉觉和和和和脑脑神神神神经损经损害，害，害，害，脑脑脊液正常脊液正常脊液正常脊液正常n n电电解解解解质质（血（血（血（血钾钾3.5)3.5)及心及心及心及心电图检查电图检查异常异常异常异常n n补钾补钾治治治治疗疗有效有效有效有效30.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.Differential diagnosisn n脊髓灰脊髓灰质炎炎(poliomyelitis)n n早期出早期出早期出早期出现现括括括括约约肌功能障碍肌功能障碍肌功能障碍肌功能障碍n n无感无感无感无感觉觉障碍障碍障碍障碍n nFever,meningealsymptoms,earlypleocytosis,and purely motor and usuallyasymmetricalareflexicparalysis.32.Differential diagnosisn n急性脊髓炎（acutemyelitis）n nTheimmediateproblemistodifferentiateGBSTheimmediateproblemistodifferentiateGBSfromacutespinalcorddisease(fromacutespinalcorddisease(markedbymarkedbysensorimotorparalysisbelowalevelonthetrunksensorimotorparalysisbelowalevelonthetrunkandsphinctericparalysis).andsphinctericparalysis).33.Clinical managementn nGeneral treatment 一般治一般治疗n nImmunotherapy 免疫治免疫治疗34.General treatmentn n保持呼吸道通保持呼吸道通保持呼吸道通保持呼吸道通畅畅n n辅助呼吸助呼吸n n密切密切密切密切观观察，察，察，察，测测肺活量肺活量肺活量肺活量20ml/kgICU20ml/kgICU20ml/kgICU20ml/kgICU必要必要必要必要时时气管气管气管气管插管，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预预防呼吸道感染防呼吸道感染防呼吸道感染防呼吸道感染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35.36.General treatmentn n预预防并防并发发症症(prevention of complication)(prevention of complication)n n坠积坠积性肺炎性肺炎性肺炎性肺炎n n褥褥褥褥疮疮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防止肢体防止肢体防止肢体防止肢体挛缩挛缩n n尿路感染尿路感染尿路感染尿路感染37.General treatmentn n预预防并防并发发症症(prevention of complication)(prevention of complication)n n合理的正合理的正合理的正合理的正压压通气、吸出分泌物通气、吸出分泌物通气、吸出分泌物通气、吸出分泌物n n经经常翻身，保持床常翻身，保持床常翻身，保持床常翻身，保持床单单平整平整平整平整n n皮下皮下皮下皮下应应用肝素用肝素用肝素用肝素n n有有有有临临床指征床指征床指征床指征时时，应应用广用广用广用广谱谱抗生素等抗生素等抗生素等抗生素等38.General treatmentn n对对症症处处理理n n必要必要必要必要时时心心心心电监护电监护n n高血高血高血高血压压小小小小剂剂量量量量受体阻滞受体阻滞受体阻滞受体阻滞剂剂n n低血低血低血低血压压补补液液液液n n心心心心动过动过速速速速通常不需要治通常不需要治通常不需要治通常不需要治疗疗n n心心心心动过缓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卡卡卡马马西平西平西平西平39.Immunotherapyn n机理机理n n抑制免疫反抑制免疫反应应，去除致病因子，去除致病因子对对神神经损经损害，使髓鞘有害，使髓鞘有时间时间再生再生n n方法方法n n血血浆浆置置换换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皮皮皮皮质质醇激素治醇激素治醇激素治醇激素治疗疗40.Plasma exchangen nTheTheusefulnessusefulnessofofplasmaplasmaexchangeexchangeininthetheevolvingevolvingphasephaseofofGBSGBS.n nInInpatientspatientswhowhoarearetreatedtreatedwithinwithin2 2weeksweeksofofonsetonset,therethereisisa areductionreductionininthetheperiodperiodofofhospitalizationhospitalizationininthethelengthlengthofoftimetimethatthepatientrequiresmechanicalventilation.thatthepatientrequiresmechanicalventilation.n nHowever,However,whenwhen plasmaplasma exchangeexchange isis delayeddelayed forfor 2 2 weeksweeks ororlongerlongerafterafterthetheonsetonsetofofthethediseasedisease,thetheprocedureprocedurehas,has,withwitha afewnotableexceptions,beenfewnotableexceptions,beenoflittlevalue.oflittlevalue.41.Plasma exchangen n血血血血浆浆置置置置换换n n机制：去除血机制：去除血机制：去除血机制：去除血浆浆中致病因子，可明中致病因子，可明中致病因子，可明中致病因子，可明显缩显缩短病程，使用越早，短病程，使用越早，短病程，使用越早，短病程，使用越早，疗疗效越效越效越效越好，好，好，好，n n专专用用用用设备设备，价格昂，价格昂，价格昂，价格昂贵贵n n适用于急性适用于急性适用于急性适用于急性进进行性加重的行性加重的行性加重的行性加重的GBSGBSn n用法：用法：用法：用法：40ml/kg40ml/kgn n禁忌症：禁忌症：禁忌症：禁忌症：严严重感染，重感染，重感染，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.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：免疫球蛋白禁忌症：免疫球蛋白禁忌症：免疫球蛋白禁忌症：免疫球蛋白过过敏，先天性敏，先天性敏，先天性敏，先天性IgAIgA缺乏缺乏缺乏缺乏n n PE PE 和和和和IVIGIVIG不必不必不必不必联联合合合合应应用用用用43.Corticosteroidsn n皮皮质类固醇固醇 n n有争有争有争有争议议n n理理理理论论上合理上合理上合理上合理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经验经验：青年人大：青年人大：青年人大：青年人大剂剂量早期使用量早期使用量早期使用量早期使用44.Corticosteroidsn nTheThevaluevalueofofcorticosteroidscorticosteroidsininthethetreatmenttreatmentofofGBSGBShashasbeenbeendisputedfordecades.disputedfordecades.n nAlthoughAlthough corticosteroidscorticosteroids cancan nono longerlonger recommendedrecommended asasroutinetreatmentforacuteGBS.routinetreatmentforacuteGBS.n nWeWe havehave observedobserved a a fewfew instancesinstances inin whichwhich thethe intravenousintravenousadministrationadministration withwith high-closehigh-close corticosteroidscorticosteroids seeminglyseeminglyhaltedtheprogressofthedisease.haltedtheprogressofthedisease.45.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 pronounced46.47.预后后n n80%患者恢复完全n n死亡率大约5%(呼吸肌麻痹)48.谢谢!49.