人体结构与功能 22-Infection in the central nervous system and Cancer of the central nervous system 学习资料

DiseasesoftheNervousSystem

KunMu（牟坤）MD,PhDDepartmentofPathologySchoolofMedicine,ShandongUniversityContentsStructureoftheNSandbasicpathologicchangesCommoncomplicationsofCNSInfectiousdiseasesofNSNeoplasmsofNSI.StructureoftheCNSandbasicpathologicchangesCerebellum(小脑)

Cerebellarpeduncles(小脑脚)Occipitallobe(枕叶)

Gyri(脑回)Sulci(脑沟)Parietallobe(顶叶)

Frontallobe(额叶)

Pons(桥脑）Fourthventricle(四脑室)

SpinalCord(脊髓)Microscopicstructureofthe

CNSNeuron（神经元）Cellbodies--greymatterCellprocesses–axons--WhitematterbundlesStainingofneuronesinthecerebralcortexMicroscopicstructureofthe

CNSNeuron（神经元）Cellbodies--greymatterCellprocesses–axons--WhitematterbundlesGlia（胶质细胞）AstrocytesOligodendrocytesEpendymalcellsStainingofastrocyteinthecerebralcortexGlia-oligodendrocytesEpendymalcellsMicroscopicstructureofthe

CNSNeuron（神经元）Cellbodies--greymatterCellprocesses–axons--WhitematterbundlesGlia（胶质细胞）AstrocytesOligodendrocytesEpendymalcellsMicroglia（小胶质细胞）Meningocyte（脑膜细胞）Vessel（血管）I.BasicpathologychangesofCNSNeuronAstrocytesOligodendrocytesEpendymalcellsMicroglia1.BasicpathologychangesoftheNeuronChromatolysis（尼氏体溶解）Redneuron（红色神经元）Simpleneuronalatrophy（单纯性神经元萎缩）Cellularinclusion（包含体形成）CellStructureproteinchange（细胞结构蛋白异常）Wallerdegeneration（Waller变性）中央型尼氏体溶解正常神经细胞1.BasicpathologychangesoftheNeuron

1.Chromatolysis（尼氏体溶解）

1.BasicpathologychangesoftheNeuron2.Redneuron（红色神经元）---Coagulationnecrosis1.BasicpathologychangesoftheNeuron1.Chromatolysis（尼氏体溶解）2.Redneuron（红色神经元）3.Simpleneuronalatrophy（单纯性神经元萎缩）Commoninsomechronicdegenerativediseases1.BasicpathologychangesoftheNeuron4.Cellularinclusion（包含体形成）Negribody-rabies（狂犬病）1.BasicpathologychangesoftheNeuron5.CellStructureproteinchange（细胞结构蛋白异常）NeurofibrillaryTangles-AZLewybodies-PD1.BasicpathologychangesoftheNeuron6.Waller

degeneration

（Waller变性）DegenerationDemyelination（脱髓鞘）RegenerationTrichrome染色：髓鞘染成红色，胶原纤维染成青色，可见髓鞘缺失、崩解。

2.Basicpathologychangesoftheastrocytes

1.Swelling（肿胀）2.Reactiveastrogliosis（反应性胶质化）

2.Basicpathologychangesoftheastrocytes2.Reactiveastrogliosis

2.Basicpathologychangesoftheastrocytes

1.Swelling（肿胀）2.Reactiveastrogliosis（反应性胶质化）3.Amyloidbody（淀粉样小体)

3.Basicpathologychangesoftheoligodendrocyte

Satellitosis（卫星现象）

4.Basicpathologychangesoftheependymalcell

Ependymalgranulation（颗粒状室管膜炎）颗粒状室管膜炎正常室管膜细胞

5.Basicpathologychangesofthemicroglia

1.Neuronophgia（噬神经细胞现象）2.Foamycell(gittercell)（格子细胞形成）3.Microglialnodules（胶质结节）

5.Basicpathologychangesofthemicroglia噬神经细胞现象neuronophagia格子细胞

gittercell5.Basicpathologychangesofthemicrogliamicroglialnodules(胶质结节）II.CommonComplicationsofCNS1.Brainedema(脑水肿)2.Hernia(脑疝)3.Hydrocephalus(脑积水)1.BrainedemaBrainedema(脑水肿):increasedwatercontentwithinthebrainparenchymaVasogenicedema:

Astateofincreasedextracellularfluidvolume;Braintumor,abscess,infarct,hemorrhageCytotoxicedema:

Theswellingofcellularelements;Hypoxia,toxication1.Brainedema1.Brainedema

脑水肿2.HerniationHerniation(脑疝形成）:Abnormalprotrusionofbraintissuethroughanopening.Causes:RaisedICP(Intracranialpressure)Intracranialexpandinglesions–tumor,haematoma,abscess2.HerniationIntracranialpressure(ICP)Withintheintactskull,thereare3majorcomponentsofintracranialpressure:thebrain,theCSFandblood.Anyincreaseinvolumeofoneofthese3componentswillproduceanincreaseinICPNormalICPis0.6-1.8kPa2.Herniation-TypesSupracallosalhernia（扣带回疝，大脑镰下疝）Tentorialhernia（小脑天幕疝，海马沟回疝）Foramenmagnumhernia(枕骨大孔疝，小脑扁桃体疝）2.Herniation扣带回疝

海马沟回疝小脑扁桃体疝

3.HydrocephalusHydrocephalus(脑积水)referstotheaccumulationofexcessiveCSFwithintheventricularsystem

3.Hydrocephalus-Types1.noncommunicatinghydrocyphalus(非交通性脑积水)

Obstructivehydrocephalus

2.communicatinghydrocyphalus(交通性脑积水)

OverproductionofCSF

DefectiveabsorptionofCSF

3.Hydrocephalus-TypesCauses:IncreasedsecrationofCSF：脉络丛肿瘤ObstractiontotheflowofCSF：肿瘤、粘连性阻塞failureofabsorptionofCSF：蛛网膜粒的闭塞Dilatedlateralventriclesseeninacoronalsection

3.Hydrocephalus-TypesIII.InfectiousdiseasesofCNS

EpidemicmeningitisEpidemicEncephalitisBRoutesofinfectionHematogenousspread

DirectimplantationTrauma,NasalorparanasalsinusesinflammationInvasionviatheperipheralnervesStructureofmengingesMeningitisAcutesuppurativemeningitis:bacteriaAcutelymphocyticmeningitis:virusChronicmeningitis:TB1.EpidemicmeningitisGroupAmeningococcusaccountsforanestimated80–85%ofallcasesinthemeningitisbelt,withepidemicsoccurringatintervalsof7–14years.Suppurativeimmflamation1.Epidemicmeningitis-TransmissionThebacteriaaretransmittedfromperson-to-personthroughdropletsofrespiratoryorthroatsecretionsfromcarriersThebacteriaoverwhelmthebody'sdefensesallowinginfectiontospreadthroughthebloodstreamtothebrain.1.Epidemicmeningitis-GrossThereare

abundantcreamy,purulentexudate,mostprominentlyoverthesuperiorsurfaceofthecerebrum.Theexudationfullinsulci.Thegyriareswollen.Thevesselsarecongested.

1.Epidemicmeningitis-Gross1.Epidemicmeningitis-MicroNeutrophilicexudate,dilatedvessels,edemainthesubarachnoidspace

1.Epidemicmeningitis-ClinicalsignsSignsofinfection(fever,

malaise,skinrashes….)Signofhighintracranialpressure

a.headacheb.vomitingSignsofmeningealirritationa.neckstiffness,episthiotonus（角弓反张）b.Kernigpositive1.Epidemicmeningitis-ClinicalsignsSignsofinfection(fever,malaise,skinrashes….)Signofhighintracranialpressure

a.headacheb.vomitingSignsofmeningealirritationa.neckstiffness,episthiotonusb.KernigpositiveCSFchanges:a.cloudypurulentb.abundantneutrophilsc.highproteinlevelandreducedglucoselevel1.Epidemicmeningitis-CSFvaluesCellsProteinGlucosePressureAcutebacterialPMNsAcuteviralLymphsNormalChronic(TB)LymphsneutrophilBacteriamonocyte1.Epidemicmeningitis-CSFvalues1.EpidemicComplications

Hydrocephalus（脑积水）Cranialnerveparalysis（颅神经麻痹）Cerebralischemiaandinfarct（脑缺血和梗死）

Fulminantmeningococcalsepticemia

暴发性脑膜炎球菌败血症anextremeformofendotoxin-inducedsepsisandcoagulopathy.Theconditionisdiagnosedclinicallybytheappearanceofhemorrhagicskinlesionsandcompromisedcirculation

Fulminantmeningococcalsepticemia

暴发性脑膜炎球菌败血症2.EpidemicEncephalitisBEpidemicencephalitisBisacuteinfectiousdiseasecausedbyencephalitisBvirusUsuallyoccursinsummer&fallThevirusistransmittedbymosquitoVirusReplicationinMononuclear-phagocyteSystem(MPS)CNSBloodStreamBloodBrainBarrierBriefViremia2.EpidemicEncephalitisB-TransmissionBloodBrainBarrier-血脑屏障2.EpidemicEncephalitisB-GrossPathologiclocation:

cerebralparenchyma2.EpidemicEncephalitisB-MicroCongestionandinflammation:endothelialcellsswelling,perivascularcuffingDegenerationandnecrosisofneurons:softingfocus,neruonophagia,satellitosisProliferationofmicroglialcells:microglialnodule2.EpidemicEncephalitisB-MicroCongestionandinflammation:endothelialcellsswelling,perivascularcuffing2.EpidemicEncephalitisB-MicroDegenerationandnecrosisofneurons:softingfocus,neruonophagia,satellitosis2.EpidemicEncephalitisB-MicroProliferationofmicroglialcells:microglialnodule2.EpidemicEncephalitisB-

ClinicalfeatureHighfeverHeadacheVomitingSleepinessConvulsion抽搐

V.TumorsofNerousSystem

TumorsofCNSPeripheralNerveTumorsTumorsofCNSComprise:10%ofalltumorsPeakincidence20-50yrCommonchildhoodtumorSupratentorialtumorsinadultsInfratentorialtumorsinchildhoodLocationdeterminesprognosisRareextraneuralmetastasisWHOGradeGradeISlowgrowingNon-malignanttumorsPatientshavelong-termsurvivalGradeIIRelativelyslowgrowingSometimesrecurashighergradetumorsMaybenon-malignantormalignant.GradeIIIMalignanttumorsOftenrecurashighergradetumorsGradeIVHighlymalignantandaggressiveTumorsofCNSGliomasMedulloblastomaMeningiomasTumorsofCNSGliomas---derivedfromglialcells,include

AstrocytomaOligodendrogliomasEpendymomaHistologicClassificationofGlialTumorsAstrocyticTumorsPilocytic(grade1)Diffuse(grade2)Anaplastic(grade3)Glioblastoma(grade4)OligodendroglialtumorsandmixedvariantsOligodendroglioma,welldifferentiated(grade2)

Anaplasticoligodendroglioma(grade3)Mixedoligodendroglioma/astrocytoma(grade2)Mixedanaplasticoligodendroglioma/astrocytoma(grade3)EpendymalTumorsMyxopapillaryependymoma(grade1)Ependymoma(grade2)Anaplasticependymoma(grade3)Astrocytoma4thto6thdecade,Cerebrum.GradingsystembasedonAnaplasiaMitoticactivityNecrosisEndothelialproliferationDiffuse(WHO-II),anaplastic(WHO-III)&Glioblastoma(WHO-IV)Molecularmarkers:GFAP,Ki67,TP53,EGFR,IDH1,MGMT…DiffuseAstrocytoma(WHO-II)Histologictype:fibrillary（纤维型）,protoplasmic（原浆型）,gemistocytic（肥胖细胞型）.Gross:apoorlydefined,gray,infiltrativetumorthatexpandsanddistortstheinvadedbrain.Thecutsurfaceofthetumoriseitherfirm,orsoftandgelatinous;cysticdegenerationmaybeseen.Micro:amildtomoderateincreaseinthenumberofglialcellnuclei,somewhatvariablenuclearpleomorphism,andtumorcellscanbeseeninfiltratingnormaltissue.GliomaCerebrumGliomaCerebrum

A.低密度细胞，核轻度异型；B.疏松瘤性间质背景中的肿瘤性纤维星形细胞；C.弥漫性微囊形成；D.不同程度的弥漫性GFAP表达。DiffuseAstrocytoma(WHO-II)

纤维型星形细胞瘤A.弥漫粘液变性；B.在微囊背景中肿瘤细胞有少许依稀可见的胞浆。DiffuseAstrocytoma(WHO-II)

原浆型星形细胞瘤肿瘤细胞浆丰富，轻度嗜酸性，核偏向周边并且GFAP强阳性。DiffuseAstrocytoma(WHO-II)

肥胖细胞型星形细胞瘤A.高密度细胞；B.大部分肿瘤细胞核内聚集TP53蛋白；C.部分区域明显的核异型和分裂活性；D.Ki67阳性率5-10%Anaplastic(WHO-III)Glioblastoma(WHO-IV)OlderadultsMediansurvival:12monthsHighlymalignantDiffuseinfiltrativetumors79岁原发性胶质母细胞瘤患者MRI。肿瘤发展迅速，初期为一个小的皮质病灶，68天后肿瘤体积明显增大，伴瘤体周围水肿和中央坏死。Glioblastoma(WHO-IV)A.具有高度间变的胶质母细胞瘤；B.腺样GBM形成腺样结构；C.GBM中少突胶质细胞成分。A.多个蜿蜒的假栅栏坏死；B.瘤细胞在坏死周围形成栅栏结构；C.TUNEL法示细胞凋亡。Glioblastoma(WHO-IV)PilocyticAstrocytoma（WHO-I)CommoninchildhoodMostslowgrowingofthegliomasSites:cerebellum,aroundIIIIVV.,opticnerveGrosslycysticwithmuralnoduleMicroscopicelongatedhair-like(pilo)cellsRosenthalfibersPilocyticAstrocytoma（WHO-I)毛细胞型星形细胞瘤年龄分布

A.毛细胞型星形细胞瘤生长侵及基底池；B.大的、多囊性小脑毛细胞型星形细胞瘤突入第四脑室，压迫脑干。C，D典型的双相成分，富有纤维，GFAP阳性区和少细胞区伴小囊形成，GFAP阴性A.术中印片示长的双极瘤细胞B.Rosenthal纤维PilocyticAstrocytoma（WHO-I)OligodendrogliomaCellsoforigin:OligodendrocytesCommonincerebralhemispheresCalcificationscommonGrades:

Lowgrade(WHO-II)

Anaplastic(WHO-III)Oligodendroglioma(WHOII)A.典型的致密分枝状血管网；B.瘤细胞胞浆透明，细胞膜明显TumorsofCNSGliomasMedulloblastomaMedulloblastoma（髓母细胞瘤）Origin:primitiveneuroectodermalcellsAge:1st－2nddecadeoflife.Mostcommonbraintumoratthisage.Medulloblastoma(髓母细胞瘤）Origin:primitiveneuroectodermalcellsAge:1st－2nddecadeoflife.Mostcommonbraintumoratthisage.Site:vermisofcerebellum,mayprojectintotheVventriculeCerebellumMedulloblastomaMedulloblastoma(髓母细胞瘤）Origin:primitiveneuroectodermalcellsAge:1st－2nddecadeoflife.Mostcommonbraintumoratthisage.Site:vermisofcerebellum,mayprojectintotheVventriculeSubarachnoiddisseminationMaycausehydrocephalusA.肿瘤转移至硬脑膜内表面；B.肿瘤转移至马尾。Medulloblastoma-SubarachnoiddisseminationA.典型的未分化肿瘤细胞片状排列；B.Homer-Wright菊形团；C.肿瘤细胞平行排列；D.产生假腺样结构的栅栏状肿瘤细胞。Medulloblastoma-HistologyMedulloblastoma-HistologyHomerWrightRosettesTumorsofCNSGliomasMedulloblastomaMeningiomasMeningiomaMeningiomasarepredominantlybenigntumorsofadultsUsuallyattachedtothedura,thatarisefromthemeningothelialcellofthearachnoidMorecommoninwomenMeningiomaGross：Meningiomasareusuallyroundedmasseswithawell-definedduralbasethatcompressunderlyingbrainbutareeasilyseparatedfromit.

Meningioma-gross矢状窦旁脑膜瘤压迫周围顶叶脑组织蝶骨中翼脑膜瘤包绕颈动脉前颅凹冠状切面显示位于嗅沟的脑膜瘤蝶骨翼的脑膜瘤斜坡脑膜瘤压迫脑干脊髓脑膜瘤压迫脊髓Meningioma-histology上皮型脑膜瘤伴核内包涵体纤维型脑膜瘤平行束状排列过渡型脑膜瘤同心圆洋葱球样结构砂粒体型脑膜瘤有大量钙化的砂粒体脊索瘤样脑膜瘤，丰富粘液背景里可见嗜伊红肿瘤细胞血管型脑膜瘤，大量血管之间为脑膜瘤细胞。V.TumorsofNerousSystem

TumorsofCNSPeripheralNerveTumorsPeripheralNerveTumorsNerveSheathTumorsNeurilemmoma（神经鞘瘤）Neurofibroma（神经纤维瘤）Perineurioma（神经束膜瘤）

MalignantPeriphe