版权说明:本文档由用户提供并上传,收益归属内容提供方,若内容存在侵权,请进行举报或认领
文档简介
CerebralAmyloidAngiopathy
脑淀粉样血管病赵元立北京天坛医院WhatisCAA?amyloiddepositionaged(>=50-60y)arteriesofthecortical,subcorticalareasM&FinincidenceRecurrent,MultipleHemorrhagePradaetal.,J.Neurosci.,2007BackgroundCerebralamyloidangiopathy(CAA)-depositionofβ-amyloidinthemediaandadventitiaofsmall-andmid-sizedarteriesICH-mostrecognizedresultof
CAARelationwithAlzheimerdiseaseCerebralAmyloidangiopathy
Two-photonprojectionofaz-seriesabout150umdeepintothebrainofaliving20-mo-oldtransgenicmouseexpressingamutanthumanamyloidprecursorprotein.Thisanimalhadamyloiddepositssurroundingsomecerebralvessels.
BrianJ.Bacskai,MassachusettsGeneralHospital,USA
EpidemiologyUnitedStates~upto15%ofallICH>60upto50%ofnontraumaticlobarICH>70~15-20per100,000population/yearaseriesof400autopsies:CAAin18.3%ofmen28%ofwomen(age40-90)aseriesof117confirmedAD:83%CAAGreenbergSM,Stroke
28(7):1418–22July1997SexandAgeSexmaybemorecommonlyinwomenincidenceofICHissameAgeagerelatedSporadicICHoccurs>60FamilialCAAatyoungeragesIcelandicform30-40,Dutch50-60DiagnosisCCheadache,vomiting,hemiplegia…PHwithouthypertension,asymptomaticPEICHrelatedfindingsCT/MRIlobar/cortical/subcorticalSAH,ventricularhemorrhage
梯度回声MR:sensitivetomicrohemorrhagePathologyCongoRed(+),Aβ(+)TransaxialT2-weightedgradient-echoMRimagesshowinnumerablemicrohemorrhagespredominantlyatcerebralgray–whitematterjunction.Microhemorrhagesarenotpresentinbasalganglia,pons,orcerebellum.LargefocalhemorrhagesarepresentinbilateralparietallobeMarisaKastoffBlitsteinAJR2007;189:720-725
GuidelinefordiagnosisBostonGroup-FourlevelsDefiniteCAA:lobar,cortical,orsubcorticalhemorrhageevidenceofsevereCAAProbableCAAwithsupportingpathologicalevidence:clinicaldata+somedegreeofvascularamyloiddepositionProbableCAA:clinicaldata+MR,nopathologicalspecimenmultiplehematomasinpatient>60PossibleCAA:patient>60clinical+MR:singlelobar,cortical,orcorticosubcorticalhemorrhage,noothercausemultiplehemorrhageswithapossiblebutnotadefinitecauseorsomehemorrhageinanatypicallocationKnudsenKA,Neurology2001;56:537–9.BhomrajThanviAgeandAgeing200635(6):565-571SpecialtypeofCAADutchtypeofhereditarycerebralhemorrhage:autosomaldominant,withmutationofamyloidprecursorprotein,atage40–60,mayproduceanabnormalanti-coagulant,whichmakeshemorrhagemorelikely.FamilialAlzheimer'sdisease:autosomaldominant,5–10%ofallADIcelandictype:autosomaldominant,withmutationinthegenecodingforcystatinC,beginat30–40withmultiplebrainhemorrhages,mostinvolvethebasalgangliaDownSyndrome:trisomy21Britishtypeoffamilialamyloidosis:autosomaldominant,associatedwithprogressivedementia,spasticity,andataxia.Brainstem,spinalcord,andcerebellumallexhibitamyloiddeposits,buthemorrhagetypicallydoesnotoccur.WhybleedingBleedingintobrainoccurastinybloodvesselscarryingamyloiddepositsbecomeheavierandmorebrittlemorelikelytoburstwithminortraumaorwithfluctuatingbloodpressureAneurysmsmaydevelop,andmayalsoruptureAmyloiddepositsmaydestroysmoothmusclecellsorcauseinflammationinthebloodvesselwall,causebloodvesseltobreakmoreeasilySethLove,FrontiersinBioscience14,4778-4792,January,2021ThecauseofamyloiddepositsinbloodvesselsinthebraininsporadicCAAisnotknownInhereditaryCAA,geneticdefects,typicallyonchromosome21,allowaccumulationofamyloid,aproteinmadeupofunitscalledbeta-pleatedsheetfibrils.Thefibrilstendtoclumptogether,sothattheamyloidcannotbedissolvedandbuildsupinthebrainbloodvesselwalls.Oneformofamyloidfibrilsubunitproteinsistheamyloidbetaprotein.StevenGreenbergGeriatricsandaging,202111(5):15-17Systemictheoryamyloidbetaproteininblooddepositedinbloodvesselsinthebrainbreakdownblood-brainbarrieramyloidbetaproteindepositedinbrainsubstanceformsneuriticplaqueSecondtheoryamyloidfibrilsproducedbyperivascularmicrogliaThirdtheorybothnervecellsandgliaproduceamyloidprecursorprotein,increaseswithaging病理机制AmyloiddamagesthemediaandadventitialeadingtothickeningofthebasalmembranestenosisofthevessellumenfragmentationoftheinternalelasticlaminaresultinfibrinoidnecrosisandmicroaneurysmformationSomeevidencesuggeststhattheamyloidisproducedinthesmoothmusclecellsofthetunicamediaasaresponsetodamageofthevesselwall(perhapsbyarteriosclerosisorhypertension)病理机制severalkeyprocessesareinvolved:productionofamyloidprecursorproteins(APP),processingofprecursorproteins,aggregationofprotein,andfibrilformation.
Impairedeliminationandaccumulationofsolubleandinsolubleβ-amyloidpeptidemayunderliethepathogenesisofCAAandexplainthelinkbetweenCAAandAD.ElectronmicroscopydemonstratesfibrilsofamyloidintheouterbasementmembraneintheinitialstageofCAAManytypesofamyloidproteinarepresentinthebody,butsomeareuniquetothebrain.β-amyloidisauniquecerebrovascularamyloidproteinAmyloidFamily:AβACysATTRAGelPrPScABriADan病理特点受累血管壁常规染色在光镜下呈不成形的,强嗜伊红的玻璃样即淀粉样改变刚果红染色呈粉红阳性物质在血管及其周围沉积,即嗜刚果红血管病脑膜及皮质中、小血管受累淀粉样物质多沉积于血管中膜及外膜血管壁增厚,管腔狭窄脑淀粉样血管病脑膜外表大血管硬化,管腔狭窄附近小动脉亦明显变性x50脑实质内可见大量淀粉样小体形成脑实质小血管管壁增厚、变性中等量淀粉样小体形成x100HEVSCongoRedPathology由皮层向皮层下过度的区域中受累血管的分布情况高倍镜下典型的嗜刚果红染色的血管壁,呈现“双环〞状标本中可见不同程度受累的血管由低倍到高倍示Aβ(+)的脑血管,集中分布在皮层及皮层下区域gradingMortalityandMorbidityCAAICHassociatedwithlowermortalityrate(11-32%)andbetterfunctionaloutcome25-40%havearecurrence,withthehighestriskinthefirstyear,associatedwithahighmortalityrate(upto40%)Cognitiveimpairmentiscommon建立标准化的微创外科诊断治疗标准〔新增样本2000例〕标准试验标准多中心大样本研究
小骨窗手术大骨瓣减压手术其它微创手段病理学检查高血压动脉硬化性淀粉样血管病疗效分析自然史
温馨提示
- 1. 本站所有资源如无特殊说明,都需要本地电脑安装OFFICE2007和PDF阅读器。图纸软件为CAD,CAXA,PROE,UG,SolidWorks等.压缩文件请下载最新的WinRAR软件解压。
- 2. 本站的文档不包含任何第三方提供的附件图纸等,如果需要附件,请联系上传者。文件的所有权益归上传用户所有。
- 3. 本站RAR压缩包中若带图纸,网页内容里面会有图纸预览,若没有图纸预览就没有图纸。
- 4. 未经权益所有人同意不得将文件中的内容挪作商业或盈利用途。
- 5. 人人文库网仅提供信息存储空间,仅对用户上传内容的表现方式做保护处理,对用户上传分享的文档内容本身不做任何修改或编辑,并不能对任何下载内容负责。
- 6. 下载文件中如有侵权或不适当内容,请与我们联系,我们立即纠正。
- 7. 本站不保证下载资源的准确性、安全性和完整性, 同时也不承担用户因使用这些下载资源对自己和他人造成任何形式的伤害或损失。
最新文档
- 红色旅游-知到答案、智慧树答案
- 2024年智能变电站自动化系统项目资金需求报告代可行性研究报告
- 2023年报纸报刊资金申请报告
- 2024年射频同轴电缆组件资金需求报告代可行性研究报告
- 2024年航天器压力控制系统项目资金筹措计划书代可行性研究报告
- 2024年工业能效管理项目资金筹措计划书
- 初级临床医学检验技士专业知识50
- 企业工作总结及领导力发展
- 我在医院工作的总结:感悟与反思
- 企业工作总结与展望报告
- 广东省中医院进修学习总结
- 变电设备检修(220kV 及以下)(变电设备检修工) 考试题库-上(单选题)
- “校长杯”校园足球联赛秩序册
- 学习能力课程
- 2023年六级口语考试历年真题整理
- 中国 2 型糖尿病防治指南(2020 年版)
- 中华民族复兴简史知到章节答案智慧树2023年华侨大学
- 测绘生产成本费用定额
- 民主测评报告民主测评报告八篇
- 二次接线电气图的基本知识讲义
- 极端天气施工方案及保证措施4篇,极端天气专项施工方案
评论
0/150
提交评论