腦白質病變專題知識講座培訓課件

腦白質病變定義腦白質病變病因血管源性白質病變白質病變與認知損害1腦白質病變專題知識講座7/26/2023腦白質病變臨床常見，多種疾病累及神經(jīng)系統(tǒng)白質分原發(fā)、繼發(fā)性兩大類.病理過程：髓鞘形成延遲、髓鞘形成障礙、脫髓鞘診斷：臨床+MRI+病理腦白質病變AnnNYAcadSci.Oct2008;1142:266–309.2腦白質病變專題知識講座7/26/2023Genetic2Demyelinative3Infectious4Inflammatory5ToxicEtiologyofCWMDisorders617891011MetabolicVascularTraumaticNeoplasticHydrocephalicDegenerative3腦白質病變專題知識講座7/26/2023GeneticDiseasesLeukodystrophies(e.g.,adrenoleukodystrophy,metachromaticleukodystrophy,globoidcellleukodystrophy)腦白質營養(yǎng)不良（腎上腺、異染性、球樣細胞腦營養(yǎng)不良）Vanishingwhitematterdisease白質消融性腦白質病Alexander’sdisease亞歷山大病Adult-onsetleukodystrophywithneuroaxonalspheroids神經(jīng)軸索球樣細胞腦白質營養(yǎng)不良Mitochondrialencephalopathywithlacticacidandstroke(MELAS)線粒體腦肌病伴乳酸酸中毒和卒中樣發(fā)作綜合征FragileXtremor-ataxiasyndrome(FXTAS)脆性X相關震顫和(或)共濟失調(diào)綜合征Aminoacidurias(e.g.,phenylketonuria)氨基酸尿（如苯丙酮尿癥）Phakomatoses(e.g.,neurofibromatosis)斑痣性錯構瘤病Mucopolysaccharidoses粘多糖貯積病Myotonicdystrophy強直性肌營養(yǎng)不良Callosalagenesis新生兒胼胝體發(fā)育不良4腦白質病變專題知識講座7/26/2023MRIofX-ALD,MLD,GLD,VWMD(A)X-連鎖的腎上腺腦白質營養(yǎng)不良(X-ALD),T1image;(B)異染性腦白質營養(yǎng)不良(MLD),FLAIRimage;(C)球形細胞腦白質營養(yǎng)不良(GLD),T2-weightedimage;(D)白質消融性腦白質病(VWMD),T1-weightedimage.X-ALD:impairedpsychomotorspeed,spatialcognition,memory,andexecutivefunctions;MLD:psychosis,behavioraldisturbances,anddementia;GLD:hemiparesis,intellectualimpairment,cerebellarataxia,andvisualfailure;AnnNYAcadSci.Oct2008;1142:266–309.5腦白質病變專題知識講座7/26/2023神經(jīng)軸索球樣細胞腦白質營養(yǎng)不良(A)FLAIRMRIintheaxialplaneshowingconfluenthighsignalintheperiventricular,deep,andsubcorticalwhitematterofthefrontalandparietallobesextendingthroughthespleniumofthecorpuscallosum.

(B)Grosspathologyofacoronalsectionofthecerebralhemisphere,showinggliosisinthecentrumsemiovale(arrow)andinternalcapsule(arrowhead).Thedisorderusuallypresentswithexecutivesystemdysfunctionandotherneurobehavioraldeficits,progressingtodementia.6腦白質病變專題知識講座7/26/2023MRIinpatientswithMELASCriteriafordiagnosisarestrokelikeepisodesbeforeage40(notconfinedtovascularterritories);encephalopathycharacterizedbyseizures,dementia,orboth;withlacticacidosisand/orragged-redfibers.Recurrentheadacheorvomitingmaybepresent.PeriventricularanddiffuseWMhyperintensities,aswellasareasofcorticalinfarctionandcerebraledema,areseenonMRI7腦白質病變專題知識講座7/26/2023脆性X相關震顫和(或)共濟失調(diào)綜合征Whitematterpallorisseeninthecerebellarparenchyma(A),aswellasinthemiddlecerebellarpeduncles(B).Progressivecognitivedeclineischaracterizedbyimpairedexecutivefunction,workingmemory,intelligence,declarativelearningandmemory,informationprocessingspeed,temporalsequencing,andvisuospatialfunctioning,butlanguageisspared.8腦白質病變專題知識講座7/26/2023DemyelinativeDiseasesMultiplesclerosisAcutedisseminatedencephalomyelitis急性播散性腦脊髓炎Acutehemorrhagicencephalomyelitis急性出血性腦脊髓炎Schilder’sdisease謝耳德氏病,彌漫性軸周性腦炎Marburg’sdisease馬爾堡病Balo’sconcentricsclerosis同心圓性硬化9腦白質病變專題知識講座7/26/2023FLAIRMRIinmultiplesclerosis(A)Whitematterhyperintensityperpendiculartothelateralventricle(Dawson’sfinger),shownbythearrow.(B)Inasecondcase,thefocalareaofhyperintensity(arrow)correspondedtotheinitialclinicalpresentationCognitiveimpairmentsinMSalsoincludeawiderangeoffocalneurobehavioralsyndromesandneuropsychiatricdisturbances10腦白質病變專題知識講座7/26/2023MRIfeaturesofADEM(A)CoronalT1-weightedpostgadoliniumimageshowingenhancinglesionsintherightmorethanlefthemispheres.(B)Axialzero-BMRIdemonstrationofthemultiplelesions.(C)FLAIRMRI6monthsaftermarkedclinicalrecoveryshowsmuchimprovedareasofhyperintensity.11腦白質病變專題知識講座7/26/2023InfectiousdiseaseHIVandAIDSdementiacomplex艾滋病癡呆綜合征Progressivemultifocalleukoencephalopathy進行性多灶性白質腦病Subacutesclerosingpanencephalitis亞急性硬化性全腦炎Progressiverubellapanencephalitis進行性風疹全腦炎Varicellazosterencephalitis水痘-帶狀皰疹病毒性腦炎Cytomegalovirusencephalitis巨細胞病毒腦炎Lymeencephalopathy萊姆腦病12腦白質病變專題知識講座7/26/2023HIVFLAIRMRIshowinghyperintensitiesinprefrontalwhitematterinapatientwithHIVandcognitiveimpairmentSchmahmannJD,PandyaDN.FiberPathwaysoftheBrain.OxfordUniversityPress;NewYork:200613腦白質病變專題知識講座7/26/2023PML(A)T2-WIshowsinvolvementofwhitematteroftherightoccipitalregion(arrow),accountingforthehemianopsiainthisHIV-positivepatient.(B)FLAIRMRIinapatientwithsystemiclymphomaandPML,demonstratingconfluentprefrontalwhitematterlesionspreadingacrossthegenuofthecorpuscallosum(arrow),andadditionallesionsaffectinglocalassociationfibersoftherightprefrontalandparieto-occipitalcortices(arrowheads).(C,D)AxialFLAIRimagesinanHIV-positivepatientshowingconfluentsubcorticalanddeepwhitematterinvolvementbyPML.Cognitivepresentationsincludefrontallobesyndromesandaphasia,progressingtoquadriparesis,mutism,andunresponsiveness14腦白質病變專題知識講座7/26/2023AutoimmuneInflammatoryDiseaseSystemiclupuserythematosus系統(tǒng)性紅斑狼瘡Behcet’sdisease白塞病,貝西氏病,貝赫切特病Sj?gren’ssyndromeWegener’sgranulomatosiTemporalarteritis顳動脈炎Polyarteritisnodosa結節(jié)性多動脈炎Scleroderma硬皮病Isolatedangiitisofthecentralnervoussystem孤立的中樞神經(jīng)系統(tǒng)血管炎Sarcoidosis結節(jié)病15腦白質病變專題知識講座7/26/2023ToxicDiseasesCranialirradiation顱腦照射Therapeuticdrugs(e.g.,methotrexate甲氨蝶呤,BCNU卡莫司汀,cyclophosphamide環(huán)磷酰胺)Drugsofabuse(e.g.,toluene甲苯,heroin海洛因)Toluene:adramaticsyndromeofdementia,ataxia,andotherneurologicsignsAlcohol(Marchiafava–Bignamidisease又稱胼胝體變性，一種慢性酒精中毒性疾病)Environmentaltoxins(e.g.,carbonmonoxide)16腦白質病變專題知識講座7/26/2023ToxicLeukoencephalopathyFLAIRMRIintheaxialplaneofapatientwithcognitivedeclineafterreceivingmethotrexateT2-WMRIappearanceintheaxialplaneoftolueneencephalopathyintwopatientsadramaticsyndromeofdementia,ataxia,andotherneurologicsigns17腦白質病變專題知識講座7/26/2023Heroin

Clinicalmanifestationsincludecerebellarmotorfindingsofataxia,dysmetriaanddysarthria,bradykinesia,rigidity,andhypophonia,andthesyndromemayprogressoverweekstopseudobulbarpalsy,akineticmutism,decorticateposturing,andspasticquadriparesis.MRIscansafterheroininhalation,knowncolloquiallyas“chasingthedragon.”FLAIRimagesintheaxialplane(A–D).Inhalationofheatedheroinvapor(colloquiallytermed“chasingthedragon”)producesadevastating,progressivespongiformleukoencephalopathy.18腦白質病變專題知識講座7/26/2023MetabolicDiseasesCobalamindeficiencyVitB12缺乏Folatedeficiency葉酸缺乏Centralpontinemyelinolysis橋腦中央髓鞘溶解癥Hypoxic–ischemicinjury缺氧缺血性腦損傷Posteriorreversibleencephalopathysyndrome可逆性后部白質腦病綜合征Hypertensiveencephalopathy/eclampsia高血壓腦病/驚厥High-altitudecerebraledema高原腦水腫19腦白質病變專題知識講座7/26/2023HypoxicischemicencephalopathyAxialMRIindelayedleukoencephalopathyafterhypoxic–ischemicinsult.(A)FLAIRimageshowsextensive,symmetricwhitematterhyperintensitieswithrelativesparingofsubcorticalwhitematter.(B)Diffusion-weightedimagingshowsrestricteddiffusionofthewhitematterabnormalities,confirmedon(C),apparentdiffusioncoefficientmapping20腦白質病變專題知識講座7/26/2023TraumaticDiseaseTraumaticbraininjury(diffuseaxonalinjury)外傷性腦損傷（彌散性軸索損傷）Shakenbabysyndrome嬰兒搖晃綜合征Corpuscallosotomy胼胝體切開術FocallesionsofWMtracts(e.g.,fornixtransection,spleniumofCCtumor)白質纖維束局灶病變（海馬傘切開術，胼胝體亞部腫瘤）21腦白質病變專題知識講座7/26/2023NeoplasticDiseasesGliomatosiscerebri腦膠質瘤Diffuselyinfiltrativegliomas彌漫性浸潤性膠質瘤Lymphomatosiscerebri大腦淋巴瘤病Langerhanscellhistiocytosis朗格漢斯細胞組織細胞增多癥Focalwhitemattertumors局灶性白質腫瘤22腦白質病變專題知識講座7/26/2023GliomatosiscerebriFIG3.55M.A,AxialT2-weightedspin-echolocalizerimage(1900/80).B,Correspondingspectrum.Stereotacticbiopsysamplewastakenfromthedenotedvoxel,withamaximumCho/NAAratioofthelesionof8.9aswellasalactatedoubletat1.35ppm.FIG4.10yearoldboywithseizure.23腦白質病變專題知識講座7/26/2023朗格漢斯細胞組織細胞增多癥T2-weightedaxialMRIinapatientwithLangerhanscellhistiocytosis,showinghyperintensesignalabnormalityinthewhitematterofthecerebellum24腦白質病變專題知識講座7/26/2023VascularDiseasesHydrocephalicDiseasesBinswanger’sdiseaseEarlyhydrocephalusCADASILNormalpressurehydrocephalusLeukoaraiosisCerebralamyloidangiopathyDegenerativeDiseaseIntravascularlymphomaWhitematterchangesinAlzheimerdiseaseWhitematterdiseaseofprematurityEffectsofagingonmyelinMigraine25腦白質病變專題知識講座7/26/2023BCAWMlesionsAging腦白質病變VascularDisease26腦白質病變專題知識講座7/26/2023白質高信號（WMHyperintensities）Leukoaraiosisisvisibleas(A)whitematterhypodensityonCTand(B)whitematterhyperintensityonFLAIRMRIinthesamepatient.27腦白質病變專題知識講座7/26/20230203040170歲以上老人，95%在MRI上可以發(fā)現(xiàn)白質高信號，隨年齡增加逐漸增多危險因素：高血壓、糖尿病、吸煙,、視網(wǎng)膜血管改變、腎臟血管改變；內(nèi)皮功能紊亂(血清同型半胱氨酸和細胞粘附分子1),血栓形成(血栓調(diào)節(jié)蛋白和纖維蛋白原）,炎性反應(CRP),氧化應激2髓鞘脫失和膠質增生，破壞髓鞘和少突膠質細胞影像學發(fā)現(xiàn)和病理學顯示的髓鞘脫失具有相關性WMH的相關概念1.JNeurolNeurosurgPsychiatry.2001Jan;70(1):9-14.2.Brain.2004Jan;127(Pt1):212-93.Neurology.2006Sep12;67(5):830-3.28腦白質病變專題知識講座7/26/202301WMH負荷與腦梗死以及卒中史相關02與腦血管病有共同的危險因素，如高血壓等03利用ACEI和利尿劑降壓治療可以減緩WMH進展04CSVD導致血管狹窄、阻塞,破壞血管的反應性，導致白質低灌注05認知障礙病人WMH高負荷，局部腦血流量下降代謝水平降低WMH的病理機制29腦白質病變專題知識講座7/26/2023腦白質病變與認知功能下降顯著相關腦白質病變越嚴重，認知功能下降越快Stroke2005;36;56-61.腦白質惡化級別無1級2級及以上初次MRI隨后的MRI年改良后的MMSE評分9594939291908988878685456789101130腦白質病變專題知識講座7/26/2023高的WMH負荷與認知功能從正常轉化到MCI，MCI轉化到癡呆有關系腦室周圍的WMH與將來的癡呆特別是AD相關在MRI上見到的白質高信號（WMH）同正電子發(fā)射斷層掃描（PET）中使用的腦淀粉樣標記物一樣，可以獨立預測AD診斷WMH與認知改變31腦白質病變專題知識講座7/26/2023WMHs影響信息加工速度和執(zhí)行功能，影響記憶的提取而不是編碼1腦室周圍白質區(qū)域為“遠端供血區(qū)”，這種情況因為缺乏吻合更易受到中度血流降低的損害2腦室周圍WMHs破壞聯(lián)系較遠皮質區(qū)域的聯(lián)系性長束，執(zhí)行功能受累較為突出3皮質下區(qū)域由致密的短回路U纖維組成，皮質下癡呆則主要是認知過程速度減慢4WMHs相關的認知改變32腦白質病變專題知識講座7/26/2023OttoBinswanger在1894年首先提出WMCs與癡呆之間可能存在關聯(lián)；Alzheimer在1902年再次提到Binswanger的病例，以后提出Binswanger腦?。˙D）這一名詞。1WMC是癡呆的危險因素，與認知功能下降有關。頭部CT檢出的AD患者WMCs可占30%，VaD患者可占70%。WMC與AD密切相關，提示血管病因素在AD病變過程中的重要性。2皮質下白質缺血主要是少突膠質細胞和髓鞘破壞；皮質下U纖維的密度較高WMLs主要破壞由弓狀U纖維組成的短的皮質-皮質聯(lián)系；腦室周圍WMLs則破壞聯(lián)系較遠皮質區(qū)域的聯(lián)系性長束3腦白質改變（WMC）與AD33腦白質病變專題知識講座7/26/20231234主要累及腦皮質小動脈、中動脈、微動脈及毛細血管，表現(xiàn)為血管壁Aβ的進行性沉積5是老年人自發(fā)性顱內(nèi)出血和認知損害的重要原因臨床表現(xiàn)主要包括：反復顱內(nèi)出血，認知下降和癡呆等約30%的腦血管淀粉樣變性患者可以出現(xiàn)老年癡呆癥狀，如嚴重的記憶障礙、注意力、定向力和計算力減退，或精神異常Ⅰ型的主要病理改變是皮質微血管內(nèi)Aβ的異常蓄積；Ⅱ型的Aβ主要沉積于腦膜血管及皮質動脈、小動脈，偶爾會累及靜脈。腦淀粉樣血管病(CAA)34腦白質病變專題知識講座7/26/2023

CAA的MRI表現(xiàn)

(A)GradientechoMRIdemonstratingmultiplepunctuateareasofhemorrhage(microbleeds,arrow)atthecortico–subcorticaljunctions.(B)MRIFLAIRsequenceinapatientwithlobarintraparenchymalhemorrhageintheleftoccipitallobe(doublearrows),aswellasperiventricularWMH(singlearrow)andsubcorticalWMH(arrowheads)35腦白質病變專題知識講座7/26/20231894年Binswanger首先報道該病例，并強調(diào)本病患者的兩個主要特點：（1）患者的腦血管有重度粥樣硬化。（2）大腦半球白質損害明顯，而皮質幾乎不受影響01深穿支動脈供血的腦室周圍白質發(fā)生大面積缺血性損害，僅有皮質穿支動脈供血的皮層下U型纖維未見明顯受損02癡呆是最顯著的癥狀，主要表現(xiàn)為記憶障礙、計算力差，可伴有心情、人格的改變，無欲、淡漠、精神錯亂03Binswanger’s腦病36腦白質病變專題知識講座7/26/2023Binswanger’sMRIHyperintensesignalabnormalityisseenatperiventricularzones,whitematterimmediatelybeneathcortex,spleniumoftheCC,andinternalandexternal/extremecapsuleregions.MultiplehypodensitiesconsistentwithlacunarinfarctsarealsoseeninthebasalgangliaandthalamusSchmahmannJDFiberPathwaysoftheBrain.OxfordUniversityPress;NewYork:200637腦白質病變專題知識講座7/26/202319號染色體notch3基因突變，血管壁增厚并含有一種嗜伊紅物質，沉積在血管中層和內(nèi)彈性膜.1MRI：多發(fā)性腔隙性腦梗死，白質灶性或彌漫性疏松，顳極區(qū)域對稱損害2請在此處輸入您的文本3CADASIL偏頭痛（＜30）、腦卒中（＞30）、癡呆及精神異常、周圍神經(jīng)亞臨床損害338腦白質病變專題知識講座7/26/2023CADASILMRI(A,B)FLAIRMRIinanasymptomatic39-year-old,notch3genepositivewithfamilyhistoryofearlystroke,whoseimagingfindingswereincidentallynoted.Characteristictemporallobewhitematterinvolvementishighlighted(arrows).(C)FLAIRMRIinapatientwithclinicallyestablishedCADASIL.(D)T2-weightedMRIinapatientwithnotch3geneandpathologicallyprovendisease.39腦白質病變專題知識講座7/26/2023神經(jīng)行為綜合征神經(jīng)精神綜合征白質性癡呆腦白質病變與神經(jīng)精神綜合征40腦白質病變專題知識講座7/26/2023ABC局部的白質病變導致失語,失用癥(apraxia),失認癥(agnosia),胼胝體分離綜合征(callosaldisconnection)等主要病因是卒中，腫瘤和脫髓鞘病也可以發(fā)生病變部位：頂下小葉、顳頂交界、前額葉、基底節(jié)、丘腦、扣帶回等，以非優(yōu)勢半球多見NeurobehavioralSyndromes41腦白質病變專題知識講座7/26/2023FocalWMlesionswithneurobehavioralmanifestations(A)Lacuneinthegenuoftherightinternalcapsule(arrow)onCTpresentingwithhemineglect.(B)DiagramoftheWMlesionresponsibleforparietalpseudothalamicpainsyndrome,thoughttodisruptthesecondsomatosensorycortexfromthalamus.(C)FLAIRMRIofposteriorreversibleencephalopathysyndromeproducingvisualloss.(D,E)FocalWMlesionconsistingofmetastaticmelanomawithsurroundingedema,