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TheroleofMRIinthediagnosisofMultipleSclerosisPublicationdateMay13,2013MS-主要討論問題典型MRI發(fā)現(xiàn)McDonald標(biāo)準(zhǔn):MRI在診斷的應(yīng)用鑒別診斷:MS-其他常見白質(zhì)病變當(dāng)我們見下面病例,首先考慮是什么病?

MS?高血壓性小血管?。炕蚱渌僖姴??

白質(zhì)腦病:許多神經(jīng)系統(tǒng)疾病在臨床和放射學(xué)均與MS相似多數(shù)意外發(fā)現(xiàn)WMLs常為血管性白質(zhì)病變鑒別診斷太難whitematterlesions(WMLs)多發(fā)性硬化特征性發(fā)現(xiàn)

胼胝體病灶和胼胝體周圍白質(zhì)病灶PDWICommon-corpuscallosumT2WIcharacteristicfinding

multiplehypointenselesionsinthecorpuscallosumT1WICommon-corpuscallosumJuxtacorticallesionsarespecificforMSinvolvementofU-fibersinMS

subcortical

lesion-alargerareaofwhitematteralmostreachingtheventriclesHypertension-

U-fibersarenotinvolvedJuxtacorticalesions高信號(hào)白質(zhì)病變與皮層間有暗帶Common-Juxtacorticallesions

adjacenttothecortexandmusttouchthecortex與皮質(zhì)接觸T2”特異性差

特征性MS灶:JuxtacorticalMSlesionlocatedintheU-fiber近皮質(zhì)灶難以與長T2皮質(zhì)區(qū)別,放大更清楚近皮質(zhì)灶腦室周多發(fā)灶,含Dawsonfinger(箭)同時(shí)有2類病灶Common-Juxtacorticallesions

Juxtacorticallesions(旁正中矢狀位更清楚)Common-Juxtacorticallesions

MS常見病灶-幕下病灶typical-infratentoriallesions注意MS典型分布多灶鄰近腦室腦干和小腦多發(fā)灶MultipleWMLswithatypicaldistributionforMS卵圓形垂直灶typical-infratentoriallesions常見幕下病灶I(lǐng)nfratentoriallesions橋腦左側(cè),右中腦角T2僅僅左側(cè)病灶增強(qiáng)增強(qiáng)disseminationintime兩個(gè)病灶,一個(gè)增強(qiáng)typical-infratentoriallesionsMS典型灶-腦室周圍白質(zhì)高信號(hào)

ahighlysensitivesequenceforlesiondetection,particularlysupratentoriallyFLAIRtypical-periventricular

lesionsmultiplelesionsinadistributioncharacteristicofMS.

PDWISpecifically,theperiventricularlesionsandthemoreperipheralwhitematterlesionsnearthegraymatter–whitematterjunctionaretypicalMRIfindingsinMStypical-periventricular

lesions多發(fā)性脊髓灶-MS另一個(gè)典型特征脊髓病灶很少見于其他CNS病,除了ADEM,Sarcoid,Lyme和SLE病灶同時(shí)見于脊髓,小腦或腦干高度提示MS!PDWI:顯示MS脊髓最佳序列:脊髓均勻低信號(hào),MS斑反差強(qiáng)更清楚相對(duì)小,周圍性,好發(fā)頸髓,小于2節(jié)段SEPDWapatientwithMStypical–SpinalcordT2WI:a27-year-oldwoman

axial:amultiplesclerosisplaquelocatedintheleftdorsolateralregionofthelefthemicord

afusiformareaofincreasedsignalintensityrepresentingaMSplaquetypical–Spinalcord非特異深白質(zhì)灶胼胝體典型MS灶:胼胝體,顳葉,近皮質(zhì),腦室旁近皮質(zhì)

CoronalPDimageofabrainspecimenwithMSinvolvement

小血管病灶僅見于額頂,少見于枕,不會(huì)在顳葉顳葉白質(zhì)病變好發(fā)于MS或CADASIL早期不見于血管病Dawsonfingers

arearadiographicfeaturedepictingdemyelinatingplaquesthroughcorpuscallosum,arrangedatrightanglesalongmedullaryveins(callososeptallocation)Theyarearelativelyspecificsignfor

MS,whichpresentsasT2hyperintensities.typical–DawsonfingersTypicalfindings

a35-year-oldmanwithrelapsingremittingMSOvoidlesionsperpendiculartotheventriclessurface

arecommonMRIrevealsmultiplelesionswithhighT2signalintensityandonelargewhitematterlesion.Thesedemyelinatinglesionsmaysometimesmimicbraintumorsbecauseoftheassociatededemaandinflammation.typical–DawsonfingersMS斑三種增強(qiáng)類型見于疾病急性(活動(dòng))期或亞急性斑塊三種增強(qiáng)類型Solid“開環(huán)征”(open-ringsign)或稱為“弓形征”(arclikesign)Ring環(huán)形solidenhancement,

TheC-shapedorarclikeenhancement,whichisfairlycharacteristicofmultiplesclerosis右顳枕增強(qiáng)斑arclikeenhancement

女,36歲,雙下肢麻木7月,無力4月多數(shù)病灶周邊輕至中度異常強(qiáng)化,呈環(huán)形(白箭頭)或開環(huán)樣強(qiáng)化(長箭頭),提示為急性或亞急性病灶FSET2WI側(cè)腦室旁深部白質(zhì)、左額皮層下多發(fā)類圓形長T2灶"FriedEgg"sign增強(qiáng)DWI上的高信號(hào)環(huán),ADC略低,提示擴(kuò)散受限,稱為“暈環(huán)征”(箭頭)左額皮層下卵圓形灶,周圍水腫,稱“煎蛋征”DWI急性期斑塊周邊的環(huán)形高信號(hào)(箭頭)ADC"Halosign"ring-likeoropenring-likeenhancementMSVariantsandDifferentialdiagnosisA39yearoldmalepresentedwithsubacuteonsetofhemianopsia.

HewasreferredforbiopsytodifferentiatebetweenagliomaordemyelinationTumefactiveMS.右顳枕瘤樣脫髓鞘病,活檢證實(shí)T2W增強(qiáng)T2WI低信號(hào)環(huán)灶周水腫占位征相對(duì)輕周圍部分增強(qiáng)(不完全環(huán))活檢處an

incompleteringMSVariantsTumefactiveMS特征MS變異型較大腦實(shí)質(zhì)灶,占位征不如其他性質(zhì)同樣大小灶增強(qiáng)周圍增強(qiáng),常呈不完全環(huán)狀,可以與表現(xiàn)為封閉環(huán)樣增強(qiáng)的膠質(zhì)瘤或腦膿腫區(qū)別部分增強(qiáng)(開放環(huán))+低信號(hào)T2環(huán)+CBF低均提示脫髓鞘瘤樣MSVariantsBalo’sConcentricSclerosis少見脫髓鞘病,脫髓鞘灶和髓鞘呈帶狀交替出現(xiàn),螺紋樣左側(cè)巨大灶T2高/等信號(hào)交替出現(xiàn)交替性線性增強(qiáng)右側(cè)較小類似灶DifferentialdiagnosisNeuromyelitisOptica脊髓腫,病變廣(3節(jié)以上)

大腦少數(shù)T2病灶診斷線索是AQP4-抗體滴度是1:1024

橫切累及大部脊髓單側(cè)視神經(jīng)炎DifferentialdiagnosisAcuteDisseminatedEncephalomyelitis(ADEM)選擇性累及皮質(zhì),基底節(jié)和丘腦廣泛皮層灰質(zhì)受累特征性丘腦灶Differentialdiagnosis不會(huì)發(fā)生在MSHereanothercaseofADEM.注意基底節(jié)受累未增強(qiáng)小腦可以增強(qiáng)DifferentialdiagnosisHereanothercaseofADEM彌漫,幕上下白質(zhì),較對(duì)稱Differentialdiagnosis模糊脊髓DifferentialdiagnosisTheMcDonaldcriteriaforMSMcDonaldcriteriaPoser-DiagnosisconclusionsThecriteriacanyieldfiveconclusions:ClinicallydefiniteMS.NeedstwoattacksandsomeclinicalorparaclinicalevidencesLaboratorysupporteddefiniteMS,showingoligoclonalbandsandclinicalorparaclinicalevidencesClinicallyprobableMS,withlessrestrictcombinations.LaboratorysupportedprobableMS.OnlytwoattacksisenoughtoenterthiscategoryNoMS–ThereisnoclinicalevicenceofhavingMS.PoserCM,,etal.

"Newdiagnosticcriteriaformultiplesclerosis:Guidelinesforresearchprotocols".AnnalsofNeurology198313(3):227–31.2001提出McDonald標(biāo)準(zhǔn),用MRI代替

原Poser標(biāo)準(zhǔn),在2005,2010修改2010年5月在愛爾蘭都柏林,國際MS診斷小組第三次會(huì)晤(2011簡(jiǎn)化版)Diagnosis2010年5月,一個(gè)國際專家小組在愛爾蘭都柏林修訂McDonaldcriteria,簡(jiǎn)化病灶空間和時(shí)間彌散標(biāo)準(zhǔn),并在某些情況下,僅一次掃描就可以確定隨著時(shí)間的推移,如果MRI顯示新病灶形成,容許MRI參與診斷,使盡早些診斷成為可能即使有了這些進(jìn)展,由于MS的復(fù)雜性和變異,仍然有些患者多年診斷不確定2011簡(jiǎn)化的修訂版使早期診斷具有高度的特異性及敏感性,讓患者更好的咨詢和早期治療2010年5月在愛爾蘭都柏林,國際MS診斷小組第三次會(huì)晤(2011簡(jiǎn)化版)進(jìn)一步修訂MS麥當(dāng)勞診斷標(biāo)準(zhǔn)。簡(jiǎn)化影像學(xué)證實(shí)CNS病變空間和時(shí)間播散,并在某些情況下,僅一次掃描就可以確定時(shí)間和空間播散保留原診斷敏感性和特異性,滿足實(shí)際應(yīng)用,更一致使用,更早診斷標(biāo)準(zhǔn)包括臨床和亞臨床實(shí)驗(yàn)室檢查,強(qiáng)調(diào)需要證明病變空間和時(shí)間播散和排除其他診斷雖然MS僅僅單靠臨床診斷,但是CNS的MRI能支持、補(bǔ)充,甚至替換某些臨床標(biāo)準(zhǔn)2011簡(jiǎn)化的修訂版使早期診斷具有高度的特異性及敏感性,讓患者更好的咨詢和早期治療disseminationinplace2005

McDonaldcriteria以及被2010版代替4條中有3條才能診斷2005McDonaldcriteria≥1T2灶至少2區(qū)域不需增強(qiáng)一次閱片:增強(qiáng)+非增強(qiáng)灶前后兩次比較:新T2灶或增強(qiáng)灶等候再次發(fā)作下面任一條可以診斷2010年5月-愛爾蘭都柏林Fordisseminationinspace(DIS)lesionsintwooutoffourtypicalareasoftheCNSarerequiredperiventricularjuxtacorticalinfratentorialspinalcordFordisseminationintime(DIT)

therearetwopossibilities:任何時(shí)間-同時(shí)存在無癥狀增強(qiáng)灶和非增強(qiáng)灶再次檢查發(fā)現(xiàn)新T2和/或增強(qiáng)灶非增強(qiáng)灶增強(qiáng)灶新T2新T2Dawsonfinger:與腦室垂直卵形灶,是與腦室表面垂直的穿透小靜脈周圍炎癥引起增強(qiáng)灶周水腫,水腫最終消退,僅留中央長T2灶增強(qiáng)和非增強(qiáng)灶同時(shí)存在腦室旁多發(fā)灶增強(qiáng)灶僅持續(xù)一月增強(qiáng)a36-year-oldwoman-relapsing-remittingMS,justabout2yearsagodisseminationintime-

Dawsonfingers增強(qiáng)的意義同時(shí)存在增強(qiáng)和非增強(qiáng)病變主要有兩層含義:證明急性炎癥病變證明疾病的時(shí)間傳播增強(qiáng)disseminationintimePeriventricular,callosal/subcallosal,

andovoidlesions胼胝體/皮質(zhì)下2個(gè)增強(qiáng)灶左腦室旁非增強(qiáng)灶T2-weightedimage4個(gè)高信號(hào)灶,3個(gè)卵圓形T1增強(qiáng)右側(cè)非增強(qiáng)低信號(hào)FrederikBarkhof,Brain(1997),120,2059–2069disseminationintime典型表現(xiàn)多發(fā)增強(qiáng)灶,均為新灶(增強(qiáng)灶僅見于一月內(nèi)),為時(shí)間播散證據(jù)許多灶近皮質(zhì),且位于U-fibersT1增強(qiáng)MS:首次發(fā)作+3月后隨訪(前后2次比較)多發(fā)增強(qiáng)灶:disseminationintime首次三月后disseminationintimeNewlesionsonT2Wimages(前后比較)僅有一個(gè)灶T2WI首次臨床發(fā)作3月后發(fā)現(xiàn)2個(gè)新灶disseminationintime Demonstrationofdisseminationintime(DIT)3monthslater滿足2005麥當(dāng)勞診斷標(biāo)準(zhǔn)第2點(diǎn)(復(fù)診至少30天發(fā)現(xiàn)新病灶和至少3月發(fā)現(xiàn)新增強(qiáng)灶),使臨床醫(yī)生能夠較早診斷MS新病灶新增強(qiáng)灶disseminationintime腦室周圍3病灶其中一個(gè)增強(qiáng)灶滿足2010麥當(dāng)勞診斷標(biāo)準(zhǔn)點(diǎn)(任何時(shí)間發(fā)現(xiàn)無癥狀新病灶和增強(qiáng)灶),使臨床醫(yī)生能夠較早診斷MSdisseminationintimeJuxtacorticallesionsinthefrontalandparietallobesT2T1增強(qiáng)其中2個(gè)增強(qiáng)FrederikBarkhof,Brain(1997),120,2059–2069disseminationintime多發(fā)近皮質(zhì)灶(箭頭)FLAIRimageT2imageMS-Diagnosis(要點(diǎn))神經(jīng)學(xué)檢查-腦脊髓損害征MRI-本身并不能確診,僅顯示可能為MS病灶CSF-支持診斷,表明腦脊髓免疫系統(tǒng)處于活動(dòng)狀態(tài)誘發(fā)電位-可協(xié)助診斷醫(yī)生-分析上述檢查和實(shí)驗(yàn)室結(jié)果,確定MS是否是實(shí)際的診斷甚至當(dāng)所有測(cè)試完成,有些人可以出現(xiàn)癥狀多年后仍然無法確診McDonald標(biāo)準(zhǔn)僅僅針對(duì)MS,如果要使用MRI診斷,必須確保病人確定是MS,不能有任何疑問而治療DiseaseModifyingAgentsFDAApprovesAgent每種藥都有副作用和風(fēng)險(xiǎn)Interferonbeta-1aweekly(Avonex)阿沃納斯Interferonbeta-1beveryotherday(Betaseron)Interferonbeta-1athreedaysaweek(Rebif)Copolymer(Copaxone)克帕松Mitoxantrone(Novantrone)Natalizumab(Tysabri)珠單抗注射液

FDAApprovesThirdOralAgentforthetreatmentofrelapsing-remittingmultiplesclerosis(MS)(2012-03)2010-芬戈莫德Fingolimod(Gilenya,Novartis諾華)2013-特立氟胺Tiflunomide(Aubagio,Genzyme/Sanofi賽諾菲)富馬酸二甲酯Mar,

2013-Dimethylfumarate(Tecfidera,BiogenIdec生物技術(shù)公司艾迪克)Theepisodecanbemonofocalormultifocalafirstneurologicepisodethatlastsatleast24hoursClinicallyisolatedsyndromecausedbyinflammation/demyelinationinoneormoresitesinCNSTheMcDonaldcriteriaforMSwererecommendedin2001byaninternationalpanelandrevisedin2005and2010AnAttackis:NeurologicaldisturbanceofkindseeninMSSubjectivereportorobjectiveobservationAtleast24hoursdurationinabsenceoffeverorinfectionExcludespseudoattacks,singleparoxysmalsymptoms(multipleepisodesofparoxysmalsymptomsoccurringover24hoursormoreareacceptableasevidence)SomehistoricaleventswithsymptomsandpatterntypicalforMScanprovidereasonableevidenceofpreviousdemyelinatingevents,evenintheabsenceofobjectivefindingsTimeBetweenAttacks:30daysbetweenonsetofevent1andonsetofevent2PositiveCSFis:OligoclonalIgGbandsinCSF(andnotserum)orelevatedIgGindex滿足TheMcDonaldcriteriaforMSThediagnosisiseither:MS:allcriteriafulfilledpossibleMS:notallcriteriafulfillednotMS:nocriteriafulfilledTheMcDonaldcriteriamakeuseoftheclinicalpresentationandtheadvancesofMRimagingWhenapatientpresentswith2ormoreattackswithclinicalevidenceof2ormoreneurologicaldeficits,thereisnoneedforadditionalrequirementstomakethediagnosisofMS,becausethereisdisseminationinplaceandtimeInallothercases(lessthan2attacksorlessthan2clinicallesions)thereisaroleforMRItofulfillthediagnosticcriteriabydemonstratingdisseminationinspace,intimeorboth.McDonald標(biāo)準(zhǔn)僅僅針對(duì)MS,如果要使用MRI診斷,必須確保病人確定是MS,不能有任何疑問而治療Coronalandmidsagittalscoutviewsareneededforreproduciblepositioningoftheslices,soyouareabletocomparefollowupstudies.

Usethecoronalscouttoplanthetruemidsagittalimageparalleltothefalxandothermidlinestructures.

Onatruemidsagittalimagealineisdrawnthroughthehypophysisandtheroofofthefourthventricle(fastigium).

ThisiscalledtheHYFA:hypophysis-fastigiumline.

Subsequentlytheslicesarepositionedwiththemiddlesliceatthelowerborderofthespleniumofthecorpuscallosum.WMLs患病率-發(fā)生率差別相當(dāng)大遺傳病:每種病盡管罕見,但作為一組疾病并非少見,但仍遠(yuǎn)比MS少Lyme‘sdisease:盡管目前流行,但仍是一個(gè)少見疾病血管病:往往是意外發(fā)現(xiàn)WMLs的病因,在所有MRI(不管何原因)中,高達(dá)50%為血管性,尤其是老人和有血管病危險(xiǎn)因素者(如動(dòng)脈硬化,高血壓,高膽固醇,糖尿病,淀粉樣血管病,高同型半胱氨酸血癥,房顫。)

Reporting如果在臨床上懷疑MS而且MR支持該診斷,那么鑒別診斷就不應(yīng)當(dāng)考慮Lyme‘s病和神經(jīng)SLE等少見病,因?yàn)檫@些少見病發(fā)病率相當(dāng)?shù)?,除非臨床表現(xiàn)支持這些少見病。Reporting

下列情況不應(yīng)將MS作為鑒別診斷臨床醫(yī)生沒有懷疑MS意外發(fā)現(xiàn)白質(zhì)腦病因?yàn)檠懿MLs發(fā)病率是MS斑的50-500倍,其概率不支持診斷MS如果臨床醫(yī)生懷疑MS,并且發(fā)現(xiàn)多個(gè)WMLs,主要鑒別是血管病,并且應(yīng)用McDonaldcriteria.DifferentialdiagnosisofWMLsWMLs鑒別診斷廣泛存在正常老人,多數(shù)為獲得性和缺血缺氧性最常見炎癥是MS最常見病毒感染是PML和HIV遺傳病常表現(xiàn)對(duì)稱性異常,必須與中毒鑒別多發(fā)灶鑒別診斷要點(diǎn)Borderzoneinfarction

單側(cè),分為內(nèi)/外分水嶺,本例為內(nèi)分水嶺區(qū),MCA/與ACAADEM

白質(zhì)和基底節(jié)多灶,感染或疫苗后,與MS同:脊髓,U纖維和胼胝體),有時(shí)增強(qiáng),與MS不用:病灶較大,兒童,單相Lyme

2-3mm病灶,與MS相似,同時(shí)有皮疹和類流感征,其他:脊髓高信號(hào),CN7增強(qiáng)Sarcoid

病灶分布酷似MS,難鑒別PML

JC病毒所致脫髓鞘病,免疫抑制患者占位,非增強(qiáng)WMLs,位于U-fibers(unlikeHIVorCMV).

可以為單側(cè),雙側(cè)不對(duì)稱更常見VirchowRobinspaces

T2WI亮,F(xiàn)LAIR黑Smallvesseldiease

位于深白質(zhì)。不位于胼胝體,近腦室或近皮質(zhì)。鑒別:multipleenhancinglesionsVasculitis好發(fā)于SLE,PAN,Behcet,syphilis,Wegener,Sjogren和PrimaryangiitisofCNS大多數(shù)為點(diǎn)狀增強(qiáng)Behcet好發(fā)土耳其人典型發(fā)現(xiàn):腦干病灶+急性期結(jié)節(jié)性增強(qiáng)Metastases灶周常有顯著水腫BorderzoneinfarctionAperipheralborderzoneinfarctionmayenhanceintheearlyphase.VirchowRobinspaces典型VRspaces(特征位置+信號(hào))位置:基底節(jié)信號(hào):所有序列信號(hào)同CSF(T1低信號(hào))T2WIFLAIR基底節(jié)多發(fā)T2高信號(hào)灶FLAIR黑色DifferentialdiagnosisofWMLsVirchowRobin腔:穿通軟腦膜支周圍含CSF的空腔常見位置:基底節(jié),三角區(qū)周圍,前連合附近,腦干中央信號(hào)特征:在所有序列與CSF相同,F(xiàn)LAIR黑色(與WMLs不同)空腔常常較?。ㄇ奥?lián)合周圍空腔例外)隨年齡增加及高血壓→血管周圍結(jié)構(gòu)萎縮→VR腔增大FLAIR

特殊病例:非常寬VR腔和融合高信號(hào)灶共存清楚顯示VR空腔與白質(zhì)病變不同白質(zhì)融合高信號(hào)寬VR腔(篩孔狀態(tài))

DifferentialdiagnosisofWMLs正常老人的發(fā)現(xiàn)在正常老人,可以發(fā)現(xiàn):PeriventricularcapsandbandsPeriventricularcaps:圍繞側(cè)腦室前后極的高信號(hào)區(qū),與myelinpallor和血管周圍腔同時(shí)存在Periventricularbandsor‘rims’:是沿著側(cè)腦室體部薄薄的線狀區(qū),與有關(guān)subependymalgliosis有關(guān)

腦輕度萎縮(腦室和腦溝增寬)深白質(zhì)斑點(diǎn)樣改變或融合灶(FazekasIandII)臨床意義尚未完全清楚一些腦血管危險(xiǎn)因素與白質(zhì)變化有關(guān),除了高血壓外,其中一個(gè)最顯著的危險(xiǎn)因素是年齡DifferentialdiagnosisofWMLs老人白質(zhì)變化白質(zhì)疏松(Leukoaraiosis,LA)放射學(xué)的用語指腦非特異性白質(zhì)改變,并沒有特定的病理學(xué)特征與之對(duì)應(yīng)常見于65歲以上老年人病理變化包括軸索喪失(lossofaxon)白質(zhì)蒼白化(myelinpallor)膠質(zhì)增生(gliosis)室管膜細(xì)胞喪失(lossofependymalcells)血管周圍間隙擴(kuò)張(enlargedperivascularspaces)DifferentialdiagnosisofWMLsTypicaldifferencesinvascularbrainstem

lesionscomparedtoMST2WI橋橫纖維中央部受累橋腦外周白質(zhì)受累,常在三叉神經(jīng)附近,或接近四腦室血管灶:腦干中央,對(duì)稱MS灶腦干周圍DifferentialdiagnosisofWMLsNormalAgingsomepunctateWMLsinthedeepwhitematterperiventricularcaps腦溝腦室擴(kuò)大深白質(zhì)點(diǎn)狀白質(zhì)改變描述深白質(zhì)改變:Fazekas分類Periventricularbands(正常)斑點(diǎn)樣深白質(zhì)改變正常輕中重融合WMLs(>75歲正常)廣泛融合WMLs(異常)病例分析:高血壓患者,發(fā)現(xiàn)多發(fā)白質(zhì)病變病灶特點(diǎn):白質(zhì)深部,不接觸腦室,不接觸皮質(zhì),不在胼胝體:不符合MS高血壓史:有利于診斷血管病感染,中毒等:臨床不支持這些病VasculardiseaseSE-T2WI病例分析:明顯是血管病

深白質(zhì)廣泛病變,U纖維和胼胝體未受累前面已經(jīng)顯示MRI50歲以上患者:腦動(dòng)脈粥樣硬化高達(dá)50%,它可見于正常血壓,但更常見于高血壓缺血性白質(zhì)腦?。罕憩F(xiàn)腔梗,分水嶺梗塞或深白質(zhì)彌散高信號(hào)遺傳性小血管病-Cadasil臨床:migraine,dementiaandfamilyhistory典型發(fā)現(xiàn)青壯年人皮質(zhì)下腔梗,伴有小囊(smallcysticlesions)和白質(zhì)腦病病灶位置有高度診斷特異性:前顳極(anteriortemporalpole)和外囊病灶位置有高度診斷特異性MRI-MostspecificfindingMostspecificfindingtodifferentiateCADASILfromischemicleukoaraiosisT2hyperintenistiesinanteriortemporalpoleAcharacteristicfindingontheMRIinpatientswithCADASILhyperintensitiesinvolvingthetemporalpolesFLAIRMRIFLAIRMRIhyperintensitiesinvolvingBilateralexternalcapsulesMRIinCADASILw/characteristicMRIfindingsofinvolvementoftheexternalcapsuleandanteriortemporallobes.

FazekasclassificationFazekasI(輕,正常)深白質(zhì)斑點(diǎn)樣改變Fazeka

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