急性炎症性脱髓鞘性-多发性神经病.pptVIP

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

IntroductionLandry-Landrysparalysis1859Landryreportedanacute,ascending,predominantlymotorparalysiswithrespiratoryfailure,leadingtodeathGuillair-Barre19162例Guillain,Barreandstrohl(1916)reportedabenignpolyneuritiswithalbuminocytologicdissociationintheCSF(raisedconcentrationofCSFproteinbutanormalcellcount)蛋白细胞分离是本病的特征

GuillainBarreLandryStrohl

IntroductionIn1956,CMillerFisherdescribedatriadofacuteophthalmoplegia,ataxia,andareflexia,nowknownasFisher’ssyndromeDuringthepast15years,GBShasbecomeclearthatthisclinicalpicture,nowcalledGuillain-Barrésyndrome,andhavedifferentpathologicalsubtypes

EpidemiologyWorldwideincidence0.6-4/100000peryearthroughouttheworldChinaincidence0.66per100000forallages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。

PathogenesisandPathophysiologyThecauseofthissyndromeisunknown,butitisgenerallyviewedtobeanautoimmuneresponsetoabacterialorviralinfection.病因尚未完全阐明

EtiologyCampylobacterJejuniEpstein-BarrVirus(EBV)?Cytomegalovirus(CMV)HIVVaccinations···········空肠肠弯曲菌

PathogenesisandPathophysiologyAnacuteimmune-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

ClinicalmanifestationsProgressiveascendingsymmetricalweaknessofthelimbsInvolvementofproximalanddistalmusclesNumbnessandtinglinginthehandsandfeetBackpain

Clinicalmanifestations

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