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1、MRIMRI在诊断在诊断MSMS的作用的作用20132013MS-主要讨论问题主要讨论问题典型MRI发现McDonald标准:MRI在诊断的应用鉴别诊断: MS-其他常见白质病变TypicalMRIfindingsinMS多发性硬化特征性发现多发性硬化特征性发现胼胝体病灶和胼胝体周围白质病灶胼胝体病灶和胼胝体周围白质病灶PDWICommon -corpus callosumT2WIcharacteristic finding multiple hypointense lesions in the corpus callosumT1WICommon -corpus callosumJuxtaco
2、rtical lesionsarespecificforMSinvolvement of U-fibers in MS subcorticalsubcortical lesion-lesion-alargerareaofwhitematteralmostreachingtheventriclesHypertension- U-fibers are not involvedJuxtacortica lesionsJuxtacortica lesions高信号白质病变与皮层间有暗带Common - Juxtacortical lesionsadjacenttothecortexandmusttou
3、chthecortex与皮质与皮质接触接触T2”特异性差特异性差 特征性特征性MSMS灶灶:Juxtacortical MS lesion located in the U-fiber近皮质灶难以与长近皮质灶难以与长T2T2皮质区别,放大更清楚皮质区别,放大更清楚近皮质灶近皮质灶 脑室周多发灶脑室周多发灶,含,含Dawson finger(Dawson finger(箭)箭)同时有同时有2 2类病灶类病灶Common - Juxtacortical lesionsCommon - Juxtacortical lesionsJuxtacortical lesions(旁正中矢状位更清楚)(旁正中
4、矢状位更清楚)Common - Juxtacortical lesionsCorticallesionsinapatientwithrelapsingremittingMSon3TMRI注意:常规序列无法注意:常规序列无法显示这些皮质灶显示这些皮质灶皮质皮质2 2个高信号灶个高信号灶3D FLAIR相应区域低信号相应区域低信号3D double-inversion recoverycross-referenced 3D T1WIMS传统认为是白质病变,近年来超高场传统认为是白质病变,近年来超高场MRI清晰显示大脑灰质也存在病灶清晰显示大脑灰质也存在病灶sometimes - Cortical
5、lesionsMS常见病灶-幕下病灶typical - infratentorial lesionsbright foci in the brainstem and cerebellumPDWI:highly sensitive for the detection of plaques in MS, especially in the posterior fossatypical - infratentorial lesions注意注意MSMS典型分布典型分布多灶邻近脑室多灶邻近脑室脑干和小脑多发灶脑干和小脑多发灶Multiple WMLs with a typical distributio
6、n for MS卵圆形垂直灶卵圆形垂直灶typical-infratentoriallesions常见幕下病灶常见幕下病灶Infratentorial lesions桥脑左侧,右中脑角T2仅仅左侧病灶增强增强dissemination in time两个病灶,一个增强两个病灶,一个增强typical-infratentoriallesionsMSMS典型灶典型灶- -脑室周围白质高信号脑室周围白质高信号 a highly sensitive sequence for lesion detection, particularly supratentoriallyFLAIRtypical - pe
7、riventricular lesionsmultiple lesions in a distribution characteristic of MS. PDWISpecifically, the periventricular lesions and the more peripheral white matter lesions near the gray matterwhite matter junction are typical MRI findings in MStypical - periventricular lesions多发性脊髓灶多发性脊髓灶- -MSMS另一个典型特征
8、另一个典型特征脊髓病灶很少见于其他脊髓病灶很少见于其他CNSCNS病,除了病,除了ADEM, Sarcoid, LymeADEM, Sarcoid, Lyme和和SLESLE病灶同时见于脊髓,小脑或病灶同时见于脊髓,小脑或脑干高度提示脑干高度提示MSMS!PDWI:显示显示MS脊髓最佳序列:脊髓均匀低信号,脊髓最佳序列:脊髓均匀低信号,MS斑反差强更清楚斑反差强更清楚 相对小,周围性,好发颈髓,小于相对小,周围性,好发颈髓,小于2 2节段节段SE PDWa patient with MStypical Spinal cord24岁,单眼视力数年后四肢运动感觉损害双下肢感觉损害就诊MS可能横切横
9、切- -背部典型三角形背部典型三角形 边界边界模糊模糊无增强效应无增强效应脊髓活动灶可增强脊髓活动灶可增强, ,但远不如脑常见但远不如脑常见边界清楚边界清楚典型病史典型病史typical Spinal cordT2WI: a 27-year-old womanaxial : a multiple sclerosis plaque located in the left dorsolateral region of the left hemicorda fusiform area of increased signal intensity representing a MS plaquetypi
10、cal Spinal cordDawson fingerstypical Dawson fingersDawson fingersarearadiographicfeaturedepictingdemyelinatingplaquesthroughcorpuscallosum,arrangedatrightanglesalongmedullaryveins(callososeptallocation)TheyarearelativelyspecificsignforMS,whichpresentsasT2hyperintensities.typical Dawson fingersTypica
11、l findingsa35-year-oldmanwithrelapsingremittingMSOvoid lesions perpendicular to the ventricles surface are common MRIrevealsmultiplelesionswithhighT2signalintensityandonelargewhitematterlesion.Thesedemyelinatinglesionsmaysometimesmimicbraintumorsbecauseoftheassociatededemaandinflammation.typical Daw
12、son fingersMSMS切片:切片:perivenous inflammation淋巴细胞浸润见于小静脉周围,这些淋巴细胞攻击髓鞘淋巴细胞浸润见于小静脉周围,这些淋巴细胞攻击髓鞘MSMS病变始于静脉周围炎症,在前病变始于静脉周围炎症,在前4 4周内周内BBBBBB受损受损初期均匀增强,可以变为环型增强初期均匀增强,可以变为环型增强Lymphocytic infiltration小静脉小静脉typical Dawson fingerssolid enhancement,The C-shaped or arclike enhancement, which is fairly characte
13、ristic of multiple sclerosis右颞枕增强斑右颞枕增强斑arclike enhancementring enhancementA39yearoldmalepresentedwithsubacuteonsetofhemianopsia.HewasreferredforbiopsytodifferentiatebetweenagliomaordemyelinationTumefactive MS. 右颞枕右颞枕瘤样脱髓鞘病,瘤样脱髓鞘病,活检证实活检证实T2W增强T2WIT2WI低信号环低信号环灶周水肿占位征相对轻占位征相对轻周围部分增强周围部分增强 ( (不完全环)不完全
14、环)活检处活检处anincomplete ringMS VariantsBalosConcentricSclerosis少见脱髓鞘病,脱髓鞘灶和髓鞘呈带状交替出现少见脱髓鞘病,脱髓鞘灶和髓鞘呈带状交替出现,螺纹样螺纹样左侧巨大灶T2高高/等信号等信号交替出现交替出现交替性线性增强右侧较小类似灶Differential diagnosisNeuromyelitis Optica脊髓肿,病变广(3节以上节以上)大脑少数T2病灶诊断线索是AQP4-抗体滴度是1:1024横切累及大部脊髓大部脊髓单侧视神经炎Differential diagnosisAcuteDisseminatedEncephalo
15、myelitis(ADEM) 选择性累及皮质,选择性累及皮质,基底节和丘脑基底节和丘脑广泛皮层灰质受累特征性丘脑灶Differential diagnosis不会发生在MSHereanothercaseofADEM.注意基底节受累未增强小脑可以增强Differential diagnosisThe McDonald criteria for MSMcDonald criteriaPoser- Diagnosis conclusionsThecriteriacanyieldfiveconclusions:Clinically definite MS.Needstwoattacksandsomec
16、linicalorparaclinicalevidencesLaboratory supported definite MS,showingoligoclonalbandsandclinicalorparaclinicalevidencesClinically probable MS,withlessrestrictcombinations.Laboratory supported probable MS.OnlytwoattacksisenoughtoenterthiscategoryNo MSThereisnoclinicalevicenceofhavingMS.PoserCM,et al
17、.Newdiagnosticcriteriaformultiplesclerosis:Guidelinesforresearchprotocols.Annals of Neurology198313(3):22731.20012001提出提出McDonaldMcDonald标准,用标准,用MRIMRI代替代替原原PoserPoser标准,在标准,在20052005,20102010修改修改2010年5月在爱尔兰都柏林,国际MS诊断小组第三次会晤(2011简化版)Diagnosis2010年5月,一个国际专家小组在爱尔兰都柏林修订McDonald criteriaMcDonald criteri
18、a,简化病灶空间和时间弥散标准,并在某些情况下,仅一次扫描就可以确定随着时间的推移,如果MRI显示新病灶形成,容许MRI参与诊断,使尽早些诊断成为可能即使有了这些进展,由于MS的复杂性和变异,仍然有些患者多年诊断不确定2011简化的修订版使早期诊断具有高度的特异性及敏感性,让患者更好的咨询和早期治疗dissemination in placedissemination in place2005Mc Donald criteria以及被以及被以及被以及被2010201020102010版代替版代替版代替版代替4 4 4 4条中有条中有条中有条中有3 3 3 3条才能诊断条才能诊断条才能诊断条才能
19、诊断2005 Mc Donald criteria1 T21 T2灶灶至少至少2 2区域区域不需增强不需增强一次阅片:增强一次阅片:增强+ +非增强灶非增强灶前后两次比较:新前后两次比较:新T2灶或增强灶灶或增强灶等候再次发作等候再次发作下面任一条可以诊断下面任一条可以诊断2010年年5月月-爱尔兰都柏林爱尔兰都柏林Fordissemination in space(DIS)lesionsintwooutoffourtypicalareasoftheCNSarerequiredperiventricularjuxtacorticalinfratentorialspinalcordFordiss
20、emination in time(DIT)therearetwopossibilities:任何时间任何时间-同时存在无症状增强灶和非增强灶再次检查再次检查发现新T2和/或增强灶非增强灶增强灶新T2新T2Dawson finger:与脑室垂直与脑室垂直卵形灶,是与卵形灶,是与脑室表面垂直的穿透小静脉周围炎症引起脑室表面垂直的穿透小静脉周围炎症引起增强灶周水肿,增强灶周水肿,水肿最终水肿最终消退,仅留中央长消退,仅留中央长T2T2灶灶增强和非增强灶同时存在增强和非增强灶同时存在脑室旁脑室旁多发灶多发灶增强灶仅持增强灶仅持续一月续一月增强增强a 36-year-old woman - relap
21、sing-remitting MS, just about 2 years agodissemination in time- Dawson fingers增强的意义 同时存在增强和非增强病变主要有两层含义:证明急性炎症病变证明疾病的时间传播增强dissemination in timePeriventricular, callosal /subcallosal, and ovoid lesions胼胝体胼胝体/ /皮质下皮质下2 2个增强灶个增强灶左脑室旁左脑室旁非增强灶非增强灶T2-weighted image4 4个高信号灶个高信号灶,3,3个卵圆形个卵圆形 T1增强右侧非增强低信号Fr
22、ederik Barkhof ,Brain (1997), 120, 20592069dissemination in time典型表现典型表现多发增强灶多发增强灶,均为新灶(增强灶仅见于一月内),为,均为新灶(增强灶仅见于一月内),为时间播散证据时间播散证据许多灶近皮质,且位于许多灶近皮质,且位于U-fibers U-fibers T1T1增强增强MS:首次发作+3月后随访(前后前后2次比较次比较)多发增强灶:多发增强灶:dissemination in time首次首次三月后三月后dissemination in timeNew lesions on T2W images (前后比较前后比
23、较)仅有一个灶仅有一个灶T2WI首次临床发作首次临床发作3 3月后月后发现发现2 2个新灶个新灶dissemination in timeDemonstration of dissemination in time (DIT)3monthslater满足满足2005麦当劳诊断标准第麦当劳诊断标准第2点(复诊至少点(复诊至少30天发现新病灶天发现新病灶和至少和至少3月发现新增强灶),使临床医生能够较早诊断月发现新增强灶),使临床医生能够较早诊断MS新病灶新病灶新增强灶新增强灶dissemination in time脑室周围3病灶其中一个增强灶满足满足2010麦当劳诊断标准点(任何时间发现无症状
24、新病灶麦当劳诊断标准点(任何时间发现无症状新病灶和增强灶),使临床医生能够较早诊断和增强灶),使临床医生能够较早诊断MSdissemination in timeJuxtacortical lesions in the frontal and parietal lobesT2 T1增强其中其中2 2个增强个增强Frederik Barkhof ,Brain (1997), 120, 20592069dissemination in time多发近皮质灶多发近皮质灶(箭头)(箭头)FLAIR imageT2 imageMS-Diagnosis(要点)神经学检查神经学检查- -脑脊髓损害征 MRI
25、-MRI-本身并不能确诊,仅显示可能为MS病灶CSF-CSF-支持诊断,表明脑脊髓免疫系统处于活动状态诱发电位诱发电位- -可协助诊断医生医生- -分析上述检查和实验室结果,确定MS是否是实际的诊断甚至当所有测试完成,有些人可以出现症状多年后仍然无法确诊McDonald McDonald 标准仅仅针对标准仅仅针对MS,MS,如果要使用如果要使用MRIMRI诊断,必须诊断,必须确保病人确定是确保病人确定是MSMS,不能,不能有任何疑问而治疗有任何疑问而治疗Disease Modifying AgentsFDA Approves Agent每种药都有副作用和风险Interferon beta-1a
26、 weekly (Avonex)Interferon beta-1a weekly (Avonex)阿沃纳斯阿沃纳斯Interferon beta-1b every other day (Betaseron) Interferon beta-1b every other day (Betaseron) Interferon beta-1a three days a week (Rebif) Interferon beta-1a three days a week (Rebif) Copolymer (Copaxone)Copolymer (Copaxone)克帕松克帕松 Mitoxantron
27、e (Novantrone) Mitoxantrone (Novantrone) Natalizumab (Tysabri)Natalizumab (Tysabri)珠单抗注射液珠单抗注射液 FDA Approves Third Oral Agent for the treatment of relapsing-remitting multiple sclerosis (MS) (2012-03)2010-芬戈莫德 FingolimodFingolimod(Gilenya, Novartis诺华 )2013-特立氟胺 TiflunomideTiflunomide(Aubagio, Genzym
28、e/Sanofi赛诺菲 ) 富马酸二甲酯Mar,2013- Dimethyl fumarateDimethyl fumarate(Tecfidera, Biogen Idec生物技术公司艾迪克 )http:/ isolated syndromecausedbyinflammation/demyelinationinoneormoresitesinCNS满足满足WMLsWMLs鉴别诊断广泛鉴别诊断广泛存在正常老人,多存在正常老人,多数为获得性和缺血数为获得性和缺血缺氧性缺氧性最常见炎症是最常见炎症是MSMS最常见病毒感染是最常见病毒感染是PMLPML和和HIVHIV遗传病常表现对称遗传病常表现对
29、称性异常,必须与中性异常,必须与中毒鉴别毒鉴别Virchow Robin spaces 典型典型VR spacesVR spaces(特征位置(特征位置+ +信号)信号)位置:位置:基底节基底节信号:信号:所有序列信号同所有序列信号同CSFCSF(T1T1低信号)低信号)T2WIFLAIR基底节多发基底节多发T2T2高信号灶高信号灶FLAIRFLAIR黑色黑色Differential diagnosis of WMLsVirchow Robin腔:穿通软脑膜支周围含CSF的空腔常见位置:基底节基底节,三角区周围,前连合附近,脑干中央信号特征:在所有序列与所有序列与CSFCSF相同相同,FLAI
30、R黑色(与WMLs不同)空腔常常较小(前联合周围空腔例外)随年龄增加及高血压血管周围结构萎缩 VR腔增大FLAIR特殊病例:特殊病例:非常宽非常宽VRVR腔和融合高信号灶共存腔和融合高信号灶共存清楚显示清楚显示VR空腔与白质病变不同空腔与白质病变不同白质白质融合高信号融合高信号宽宽VR腔(筛孔状态)腔(筛孔状态)Differential diagnosis of WMLs正常老人的发现正常老人的发现在正常老人,可以发现在正常老人,可以发现: : Periventricular caps and bandsPeriventricular caps and bandsPeriventricular
31、 capsPeriventricular caps:围绕侧脑室前后极的高信号区,与:围绕侧脑室前后极的高信号区,与myelin myelin pallorpallor和血管周围腔同时存在和血管周围腔同时存在Periventricular bandsPeriventricular bands or rims or rims :是沿着侧脑室体部薄薄的线:是沿着侧脑室体部薄薄的线状区,与有关状区,与有关subependymal gliosissubependymal gliosis有关有关 脑轻度萎缩(脑室和脑沟增宽)脑轻度萎缩(脑室和脑沟增宽)深白质斑点样改变或融合灶(深白质斑点样改变或融合灶(F
32、azekas I and IIFazekas I and II)临床意义尚未完全清楚临床意义尚未完全清楚一些脑血管危险因素与白质变化有关,除了高血压外,一些脑血管危险因素与白质变化有关,除了高血压外,其中一个最显著的危险因素是年龄其中一个最显著的危险因素是年龄Differential diagnosis of WMLsNormal AgingsomepunctateWMLsinthedeepwhitematterperiventricular caps脑沟脑室扩大深白质点状白质改变描述深白质改变:描述深白质改变:FazekasFazekas分类分类Periventricularbands(正常
33、)斑点斑点样深白质改变样深白质改变正常正常轻轻中中重重融合融合WMLs(75岁正常)岁正常)广泛融合广泛融合WMLs(异常)(异常)遗传性小血管病遗传性小血管病- -Cadasil临床:临床:migraine, dementia and family historymigraine, dementia and family history典型发现典型发现青壮年人青壮年人皮质下腔梗,伴有小囊(皮质下腔梗,伴有小囊(small cystic lesionssmall cystic lesions)和白质脑病)和白质脑病病灶位置有高度诊断特异性:病灶位置有高度诊断特异性:前颞极前颞极(anterio
34、r temporal poleanterior temporal pole)和外)和外囊囊病灶位置有高度诊断特异性病灶位置有高度诊断特异性MRI-MostspecificfindingMostspecificfindingtodifferentiateCADASILfromischemicleukoaraiosisT2hyperintenistiesinanteriortemporalpoleA characteristic finding on the MRI in patients with CADASILhyperintensitiesinvolvingthetemporalpolesF
35、LAIRMRIFLAIRMRIhyperintensitiesinvolvingBilateralexternalcapsulesMRIinCADASILw/characteristicMRIfindingsofinvolvementoftheexternalcapsuleandanteriortemporallobes.Fazekas classificationFazekas IFazekas I(轻,正常(轻,正常)深白质深白质斑点样斑点样改变改变Fazekas IIFazekas II(中)(中)大片大片WMLsWMLs开始融合开始融合(7575岁异常)岁异常)Fazekas IIIF
36、azekas III(重)、(重)、广泛融合广泛融合WMLsWMLs(任何年(任何年龄为异常)龄为异常)这些白质变化可能由微这些白质变化可能由微血管病引起,更常见于血管病引起,更常见于有血管病危险因素患者有血管病危险因素患者描述深白质的变化描述深白质的变化Formsofwhitematterlesions(WML)small capslarge capsextending capsthin liningsmoothhaloirregular periventricular WMLpunctuate deep WMLdeep WML beginning confluenceconfluent deep WML