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,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 IntroductionlLandry-Landrys paralysis 1859lLandry reported an acute,ascending,predominantly motor paralysis with respiratory failure,leading to deathlGuillair-Barre 1916 2例 lGuillain,Barre an
2、d strohl(1916)reported a benign polyneuritis with albuminocytologic dissociation in the CSF(raised concentration of CSF protein but a normal cell count)蛋白细胞分离蛋白细胞分离是本病的特征GuillainBarre LandryStrohlIntroductionlIn 1956,C Miller Fisher described a triad of acute ophthalmoplegia,ataxia,and areflexia,now
3、 known as Fishers syndromelDuring the past 15 years,GBS has become clear that this clinical picture,now called Guillain-Barr syndrome,and have different pathological subtypes EpidemiologylWorldwide incidencel0.6-4/100 000 per year throughout the worldlChina incidencel0.66 per 100 000 for all agesl可发
4、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l儿童和青少年，夏初。lEMG：轴索损害，AMAN。lEMG符合AMAN的为65，符合AIDP的为24。l66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。临床表现：中国临床表现：中国l病理：lAMAN：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。lAIDP：IgG和补体在髓鞘外沉积，巨噬细胞也在髓鞘外，“撕开”髓鞘。lAMSAN：感觉轴索比运动轴索损害重
5、。lEMG不能预测病理。Pathogenesis and PathophysiologylThe cause of this syndrome is unknown,but it is generally viewed to be an autoimmune response to a bacterial or viral infection.l病因尚未完全阐明病因尚未完全阐明EtiologylCampylobacter JejunilEpstein-Barr Virus(EBV)lCytomegalovirus(CMV)lHIVlVaccinationsl空肠肠弯曲菌空肠肠弯曲菌Pathog
6、enesis and PathophysiologylAn acute immune-mediated polyneuropathy,component of pathogen was similar with myelin sheath of peripheral nervel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周围神经的髓鞘成分相似周围神经的髓鞘成分相似 Pathophysiologyl主要病理特点主要病理特点(principal characteristic of pathology)l节段性脱髓鞘节段性脱髓鞘(segmental dem
7、yelization)l小血管周围炎性细胞浸润小血管周围炎性细胞浸润Clinical manifestationsl多数患者有前驱症状多数患者有前驱症状(起病前起病前13周）周）l呼吸道感染症状呼吸道感染症状l喉痛、鼻塞、发热喉痛、鼻塞、发热l消化道症状消化道症状l腹泻、呕吐腹泻、呕吐Clinical manifestationslProgressive ascending symmetrical weakness of the limbslInvolvement of proximal and distal muscleslNumbness and tingling in the hands
8、 and feetlBack painClinical manifestationslDepressed or absent reflexeslInvolvement of cranial nerves(facial nerves most commonly involved)lRespiratory failure(involved respiratory muscles)lProgression to peak disability in 4 wklautonomic nerve symptom AssessmentlCerebrospinal fluidlIncreased protei
9、n usually after 7 to 10 days.lWhile some protein is normally present,an increased amount without an increase in the number of white blood cells may indicate GBSl蛋白细胞分离AssessmentlNerve conduction velocity testl Nerve conduction studies are a dependable and early diagnostic indicator of GBS.lshows dem
10、yelization and damage to the nerve sheathlF反应、H反射异常 PL延长，NCV减慢l 传导阻滞现象，伴或不伴有波幅降低Assessmentl腓肠神经活检腓肠神经活检l节段性脱髓鞘节段性脱髓鞘l小血管周围炎性细胞浸润小血管周围炎性细胞浸润lElectrocardiogram(EKG)lMay show abnormalities in cardiac rhythml心律失常Subtypes of GBS l经典型经典型 AIDPlFisher综合症综合症(Miller Fisher syndrome)：l三联征三联征-“眼外肌麻痹眼外肌麻痹、共济失调、腱
11、反射消失共济失调、腱反射消失”，还还有中枢神经系统损害有中枢神经系统损害 lIt was thought to be a variant of GBS and comprise complete ophthalmoplegia with ataxia and are flexial脑神经型脑神经型Subtypes of GBS l轴突型轴突型 l纯运动型（纯运动型（AMAN）l运动运动 感觉感觉 型型（AMSAN）l急性感觉性多发性神经炎（急性感觉性多发性神经炎（ASP）l急性全自主神经病（急性全自主神经病（APN）l假性肌营养不良假性肌营养不良l复发型复发型DiagnosislRequire
12、d for diagnosislProgressive weakness of one or more limblDistal areflexia with proximal areflexia or hyporeflexiaDiagnosislSupportive diagnosislProgression of symptoms over days to 4 wklRelative symmetry of deficits lMild sensory involvementlCranial nerve involvement(especially VII)lRecovery beginni
13、ng within 4 wkDiagnosislSupportive diagnosislAutonomic dysfunctionlNo fever lIncreased CSF protein after 1 wklCSF white blood cell count 10/LlNerve conduction slowing or blocked by several weeksDiagnosislAgainst diagnosislSignificant asymmetric weaknesslBowel or bladder dysfunction at onset or persi
14、stentlCSF white blood cell count 50 or PMN count 0LlWell-demarcated sensory levelDiagnosislExcluding diagnosislIsolated sensory involvement,without weaknesslAnother polyneuropathy that explains clinical pictureDifferential diagnosislAcquired hypokalemialBotulismlMyasthenia gravislPeriodic paralysisl
15、PoliomyelitislPolymyositislTick paralysislDiphtherialTransverse myelitislHeavy metal(lead and arsenic poisoning)Differential diagnosisl低钾性周期性瘫痪低钾性周期性瘫痪(hypokalemic periodic paralysis)l无病前感染史，常有发作史无病前感染史，常有发作史l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l电解质（血钾电解质（血钾3.5)及心电图检查异常及心电图检查异常l补钾治疗有效补钾治疗有效Differential di
16、agnosisl重症肌无力重症肌无力(myasthenia gravis)l骨骼肌骨骼肌 病态易疲劳性、波动性病态易疲劳性、波动性lno sensory symptoms ltendon reflexes are unimpaired Differential diagnosisl脊髓灰质炎脊髓灰质炎(poliomyelitis)l早期出现括约肌功能障碍早期出现括约肌功能障碍l无感觉障碍无感觉障碍l Fever,meningeal symptoms,early pleocytosis,and purely motor and usually asymmetrical areflexic par
17、alysis.Differential diagnosisl急性脊髓炎（acute myelitis）lThe immediate problem is to differentiate GBS from acute spinal cord disease(marked by sensorimotor paralysis below a level on the trunk and sphincteric paralysis).Clinical managementlGeneral treatment 一般治疗一般治疗lImmunotherapy 免疫治疗免疫治疗General treatme
18、ntl保持呼吸道通畅保持呼吸道通畅l辅助呼吸辅助呼吸l密切观察，测肺活量密切观察，测肺活量20ml/kgICU20ml/kgICU必要时气管插管，必要时气管插管，使用呼吸器使用呼吸器l预防呼吸道感染预防呼吸道感染l翻身、拍背、稀化痰液、吸痰翻身、拍背、稀化痰液、吸痰General treatmentl预防并发症(prevention of complication)l坠积性肺炎坠积性肺炎l褥疮褥疮l血栓性静脉炎血栓性静脉炎l防止肢体挛缩防止肢体挛缩l尿路感染尿路感染General treatmentl预防并发症(prevention of complication)l合理的正压通气、吸出分泌物
19、合理的正压通气、吸出分泌物l经常翻身，保持床单平整经常翻身，保持床单平整l皮下应用肝素皮下应用肝素l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 treatmentl对症处理l必要时心电监护必要时心电监护l高血压高血压小剂量小剂量受体阻滞剂受体阻滞剂l低血压低血压补液补液l心动过速心动过速通常不需要治疗通常不需要治疗l心动过缓心动过缓阿托品阿托品l疼痛疼痛卡马西平卡马西平Immunotherapyl机理l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l方法l血浆置换l静脉注射免疫球蛋白静脉注射免疫球蛋白l皮质醇激素治疗皮质醇激素治疗Plasma excha
20、ngelThe usefulness of plasma exchange in the evolving phase of GBS.lIn patients who are treated within 2 weeks of onset,there is a reduction in the period of hospitalization in the length of time that the patient requires mechanical ventilation.lHowever,when plasma exchange is delayed for 2 weeks or
21、 longer after the onset of the disease,the procedure has,with a few notable exceptions,been of little value.Plasma exchangel血浆置换血浆置换l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，l专用设备，价格昂贵专用设备，价格昂贵l适用于急性进行性加重的适用于急性进行性加重的GBSl用法：用法：40ml/kgl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，凝血功能障
22、碍凝血功能障碍Intravenous immunoglobulinl静脉注射免疫球蛋白静脉注射免疫球蛋白l尽早施行尽早施行l用法：用法：0.4g/(kg.d)5天天l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IgA缺乏缺乏l PE 和和IVIG不必联合应用不必联合应用Corticosteroidsl皮质类固醇皮质类固醇 l有争议有争议l理论上合理理论上合理l研究表明无效研究表明无效l经验：青年人大剂量早期使用经验：青年人大剂量早期使用CorticosteroidslThe value of corticosteroids in the treatment of GBS has
23、 been disputed for decades.lAlthough corticosteroids can no longer recommended as routine treatment for acute GBS.lWe have observed a few instances in which the intravenous administration with high-close corticosteroids seemingly halted the progress of the disease.PrognosislPrognosislThe majority of patients recover completely or nearly completely lIn about 10 percent of patients,the residual disability is pronounced预后预后l80%患者恢复完全l死亡率大约5%(呼吸肌麻痹)
