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AcuteInflammatoryDemyelinatingPolyneuropathy AIDP 浙江大学医学院附属第一医院 Introduction Landry Landry sparalysis1859Landryreportedanacute ascending predominantlymotorparalysiswithrespiratoryfailure leadingtodeathGuillair Barre19162例Guillain Barreandstrohl 1916 reportedabenignpolyneuritiswithalbuminocytologicdissociationintheCSF raisedconcentrationofCSFproteinbutanormalcellcount 蛋白细胞分离是本病的特征 Guillain Barre Landry Strohl Introduction In1956 CMillerFisherdescribedatriadofacuteophthalmoplegia ataxia andareflexia nowknownasFisher ssyndromeDuringthepast15years GBShasbecomeclearthatthisclinicalpicture nowcalledGuillain Barr syndrome andhavedifferentpathologicalsubtypes Epidemiology Worldwideincidence0 6 4 100000peryearthroughouttheworldChinaincidence0 66per100000forallages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 PathogenesisandPathophysiology Thecauseofthissyndromeisunknown butitisgenerallyviewedtobeanautoimmuneresponsetoabacterialorviralinfection 病因尚未完全阐明 Etiology CampylobacterJejuniEpstein BarrVirus EBV Cytomegalovirus CMV HIVVaccinations 空肠肠弯曲菌 PathogenesisandPathophysiology Anacuteimmune mediatedpolyneuropathy 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 principalcharacteristicofpathology 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 ProgressiveascendingsymmetricalweaknessofthelimbsInvolvementofproximalanddistalmusclesNumbnessandtinglinginthehandsandfeetBackpain Clinicalmanifestations DepressedorabsentreflexesInvolvementofcranialnerves facialnervesmostcommonlyinvolved Respiratoryfailure involvedrespiratorymuscles Progressiontopeakdisabilityin4wkautonomicnervesymptom Assessment CerebrospinalfluidIncreasedproteinusuallyafter7to10days Whilesomeproteinisnormallypresent anincreasedamountwithoutanincreaseinthenumberofwhitebloodcellsmayindicateGBS蛋白细胞分离 Assessment NerveconductionvelocitytestNerveconductionstudiesareadependableandearlydiagnosticindicatorofGBS showsdemyelizationanddamagetothenervesheathF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AIDPFisher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ItwasthoughttobeavariantofGBSandcomprisecompleteophthalmoplegiawithataxiaandareflexia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 RequiredfordiagnosisProgressiveweaknessofoneormorelimbDistalareflexiawithproximalareflexiaorhyporeflexia Diagnosis SupportivediagnosisProgressionofsymptomsoverdaysto4wkRelativesymmetryofdeficitsMildsensoryinvolvementCranialnerveinvolvement especiallyVII Recoverybeginningwithin4wk Diagnosis SupportivediagnosisAutonomicdysfunctionNofeverIncreasedCSFproteinafter1wkCSFwhitebloodcellcount 10 LNerveconductionslowingorblockedbyseveralweeks Diagnosis AgainstdiagnosisSignificantasymmetricweaknessBowelorbladderdysfunctionatonsetorpersistentCSFwhitebloodcellcount 50orPMNcount 0 LWell demarcatedsensorylevel Diagnosis ExcludingdiagnosisIsolatedsensoryinvolvement withoutweaknessAnotherpolyneuropathythatexplainsclinicalpicture Differentialdiagnosis AcquiredhypokalemiaBotulismMyastheniagravisPeriodicparalysisPoliomyelitis PolymyositisTickparalysisDiphtheriaTransversemyelitisHeavymetal 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nosensorysymptomstendonreflexesareunimpaired Differentialdiagnosis 脊髓灰质炎 poliomyelitis 早期出现括约肌功能障碍无感觉障碍Fever meningealsymptoms earlypleocytosis andpurelymotorandusuallyasymmetricalareflexicparalysis Differentialdiagnosis 急性脊髓炎 acutemyelitis TheimmediateproblemistodifferentiateGBSfromacutespinalcorddisease 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 Generaltreatment 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 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 TheusefulnessofplasmaexchangeintheevolvingphaseofGBS Inpatientswhoaretreatedwithin2weeksofonset thereisareductionintheperiodofhospitalizationinthelengthoftimethatthepatientrequiresmechanicalventilation However whenplasmaexchangeisdelayedfor2weeksorlongeraftertheonsetofthedisease theprocedurehas withafewnotableexceptions beenoflittlevalue Plasmaexchange 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 ThevalueofcorticosteroidsinthetreatmentofGBShasbeendisputedfordecades AlthoughcorticosteroidscannolongerrecommendedasroutinetreatmentforacuteGBS Wehaveobse