神經(jīng)系統(tǒng)病理NERVOUSSYSTEM月(神經(jīng)模塊講義七制中英文)

神經(jīng)系統(tǒng)疾病ChenMaohuaiDept.ofPathology,ShantouUniversityMedicalCollege當(dāng)前第1頁\共有164頁\編于星期四\20點(diǎn)第一節(jié)神經(jīng)系統(tǒng)疾病的基本病變當(dāng)前第2頁\共有164頁\編于星期四\20點(diǎn)NEURONS當(dāng)前第3頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第4頁\共有164頁\編于星期四\20點(diǎn)一、神經(jīng)元的基本病變

（一）中央性Nissl小體溶解神經(jīng)細(xì)胞腫脹，核偏位，Nissl小體僅見于細(xì)胞周邊部分。是可逆性變化。見于病毒感染，VB缺乏，壞血病及神經(jīng)元與軸突斷離時(shí)。

當(dāng)前第5頁\共有164頁\編于星期四\20點(diǎn)Nisslbodylysis當(dāng)前第6頁\共有164頁\編于星期四\20點(diǎn)（二）神經(jīng)元急性壞死（紅色神經(jīng)元）神經(jīng)細(xì)胞核固縮，胞體縮小變形。Nissl小體消失，胞漿深染呈伊紅色，稱為紅色神經(jīng)細(xì)胞。

當(dāng)前第7頁\共有164頁\編于星期四\20點(diǎn)紅色神經(jīng)元當(dāng)前第8頁\共有164頁\編于星期四\20點(diǎn)鬼影細(xì)胞（ghostcell）：神經(jīng)細(xì)胞壞死后細(xì)胞核溶解消失，胞漿淡染，隱約可見細(xì)胞輪廓的死亡細(xì)胞。常見于大腦皮層的錐體細(xì)胞和小腦蒲肯野氏細(xì)胞。當(dāng)前第9頁\共有164頁\編于星期四\20點(diǎn)（三）神經(jīng)元慢性病變：一組特殊的病變1.單純性神經(jīng)元萎縮神經(jīng)元慢性漸進(jìn)性變性以致死亡的過程。當(dāng)前第10頁\共有164頁\編于星期四\20點(diǎn)2.神經(jīng)元纖維纏結(jié)(細(xì)胞結(jié)構(gòu)蛋白異常)神經(jīng)原纖維變粗在胞核周圍凝結(jié)卷曲呈纏結(jié)狀。鍍銀染色陽性，電鏡下為7-10nm雙螺旋微絲。為神經(jīng)元趨向死亡的標(biāo)志。常見于Alzheimer病等。當(dāng)前第11頁\共有164頁\編于星期四\20點(diǎn)3.特殊胞漿包含體Lewy小體―――見于病毒感染和變性疾病。如Parkinson病的黑質(zhì)、藍(lán)斑等處的神經(jīng)細(xì)胞胞漿內(nèi)所見的圓形均質(zhì)弱嗜堿性包含體，周圍可見空暈。Negri小體―――見于狂犬病時(shí)海馬和皮層的神經(jīng)細(xì)胞漿中（具有診斷價(jià)值）。當(dāng)前第12頁\共有164頁\編于星期四\20點(diǎn)二、神經(jīng)纖維的基本病變（一）Waller變性指神經(jīng)纖維被切斷后，軸索與神經(jīng)元胞體斷離，其遠(yuǎn)端和部分近端軸索及其所屬髓鞘發(fā)生變性、崩解和被吞噬細(xì)胞吞噬的過程。當(dāng)前第13頁\共有164頁\編于星期四\20點(diǎn)過程：①軸索變性②髓鞘脫失③細(xì)胞增生反應(yīng)當(dāng)前第14頁\共有164頁\編于星期四\20點(diǎn)（二）脫髓鞘樣變由于雪旺細(xì)胞（Schwann）變性或髓鞘損傷導(dǎo)致髓鞘板層分離腫脹，斷裂崩解成脂質(zhì)小滴進(jìn)而完全消失。軸索相對(duì)保留。繼發(fā)性脫髓鞘―――創(chuàng)傷、感染、缺氧等原因引起的脫髓鞘。白質(zhì)營養(yǎng)不良―――某些遺傳性髓鞘合成障礙性疾病。當(dāng)前第15頁\共有164頁\編于星期四\20點(diǎn)三、神經(jīng)膠質(zhì)細(xì)胞的基本病變神經(jīng)膠質(zhì)細(xì)胞包括星形膠質(zhì)細(xì)胞、少突膠質(zhì)細(xì)胞、室管膜細(xì)胞（一）星形膠質(zhì)細(xì)胞病變當(dāng)前第16頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第17頁\共有164頁\編于星期四\20點(diǎn)1、星形膠質(zhì)細(xì)胞增生（反應(yīng)性膠質(zhì)化與膠質(zhì)瘢痕）纖維型星形膠質(zhì)細(xì)胞增生→膠質(zhì)瘢痕（GFAP+++）膠質(zhì)瘢痕與纖維瘢痕不同之處在于星形膠質(zhì)細(xì)胞不產(chǎn)生膠原纖維及相應(yīng)間質(zhì)蛋白。見于缺氧、感染、中毒等腦損傷時(shí)。

當(dāng)前第18頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第19頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第20頁\共有164頁\編于星期四\20點(diǎn)膠質(zhì)細(xì)胞增生結(jié)節(jié)當(dāng)前第21頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第22頁\共有164頁\編于星期四\20點(diǎn)2、星形膠質(zhì)細(xì)胞肥大星形膠質(zhì)細(xì)胞呈現(xiàn)體積增大、胞漿豐富、伊紅色、核偏位。電鏡下胞漿中充滿線粒體，內(nèi)質(zhì)網(wǎng)、溶酶體、膠質(zhì)纖維等細(xì)胞器。這種肥胖星形膠質(zhì)細(xì)胞常見于局部缺氧、水腫、梗死、腫脹、腫瘤周圍。當(dāng)前第23頁\共有164頁\編于星期四\20點(diǎn)（二）少突膠質(zhì)細(xì)胞病變表現(xiàn)為髓鞘改變，白質(zhì)營養(yǎng)不良（髓鞘形成不良）和脫髓鞘疾病。缺氧中毒時(shí)表現(xiàn)為急性腫脹核周空暈——自溶變化。當(dāng)前第24頁\共有164頁\編于星期四\20點(diǎn)衛(wèi)星現(xiàn)象（satellitosis）：神經(jīng)元胞體周圍被5個(gè)或5個(gè)以上少突膠質(zhì)細(xì)胞圍繞稱為衛(wèi)星現(xiàn)象。當(dāng)前第25頁\共有164頁\編于星期四\20點(diǎn)衛(wèi)星現(xiàn)象當(dāng)前第26頁\共有164頁\編于星期四\20點(diǎn)(三)室管膜細(xì)胞各種致病因素引起局部室管膜細(xì)胞丟失，室膜下星形膠質(zhì)細(xì)胞增生充填，形成突向腦室面的細(xì)小顆粒，稱為顆粒性室管膜炎。巨細(xì)胞病毒感染可引起室管膜細(xì)胞廣泛損傷，殘留細(xì)胞內(nèi)可見包含體。

當(dāng)前第27頁\共有164頁\編于星期四\20點(diǎn)(四)小膠質(zhì)細(xì)胞小膠質(zhì)細(xì)胞屬于單核巨噬細(xì)胞系統(tǒng)對(duì)損傷的反應(yīng)：(1)增生形成膠質(zhì)結(jié)節(jié)：小膠質(zhì)細(xì)胞呈局灶性增生，聚集成團(tuán)。常見于病毒感染時(shí)。(2)增生呈桿狀細(xì)胞(rodcell)：胞體變窄，胞突減少呈雙極桿狀。(3)噬神經(jīng)細(xì)胞現(xiàn)象（neuronophagia）：壞死的神經(jīng)元被增生的小膠質(zhì)細(xì)胞或巨噬細(xì)胞吞噬的過程。(4)格子細(xì)胞(gittercell)也稱泡沫細(xì)胞（foamycell）：小膠質(zhì)細(xì)胞或巨噬細(xì)胞吞噬神經(jīng)組織崩解產(chǎn)物后，胞體增大，含大量脂滴，HE染色呈空泡狀，蘇丹Ⅲ染色陽性。當(dāng)前第28頁\共有164頁\編于星期四\20點(diǎn)噬神經(jīng)元現(xiàn)象當(dāng)前第29頁\共有164頁\編于星期四\20點(diǎn)第二節(jié)

中樞神經(jīng)系統(tǒng)疾病常見的并發(fā)癥

當(dāng)前第30頁\共有164頁\編于星期四\20點(diǎn)一、顱內(nèi)壓升高及腦疝形成側(cè)臥位的腦脊液壓超過2kPa（正常為0.6～0.8kPa）即為顱內(nèi)壓增高?？梢鹉X移位、腦室變形、腦疝形成（herniation）。

當(dāng)前第31頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第32頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第33頁\共有164頁\編于星期四\20點(diǎn)1．扣帶回疝又稱大腦鐮下疝（Subfalcineherniation）

是因一側(cè)大腦半球特別是額、頂、顳葉的血腫或腫瘤等占位性病變，引起中線向?qū)?cè)移位，同側(cè)扣帶回從大腦鐮的游離邊緣向?qū)?cè)膨出，形成扣帶回疝。

當(dāng)前第34頁\共有164頁\編于星期四\20點(diǎn)2．小腦天幕疝又稱海馬鉤回疝(Transtentorialherniation，uncinateherniation)位于小腦天幕以上的額葉或顳葉內(nèi)側(cè)的腫瘤、出血、梗死等病變引起腦組織體積腫大，導(dǎo)致顳葉的海馬鉤回經(jīng)小腦天幕孔向下膨出。當(dāng)前第35頁\共有164頁\編于星期四\20點(diǎn)3．小腦扁桃體疝又稱枕骨大孔疝（Tonsillarherniation）主要由于顱內(nèi)高壓或后顱凹占位性病變將小腦和延髓推向枕骨大孔并向下移位而形成小腦扁桃體疝。當(dāng)前第36頁\共有164頁\編于星期四\20點(diǎn)二、腦水腫腦組織中由于液體過多貯積而形成腦水腫（brainedema）。1．血管源性腦水腫最為常見，是血管通透性增加的結(jié)果。2．細(xì)胞毒性腦水腫多見于缺血或中毒引起的細(xì)胞損害。當(dāng)前第37頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第38頁\共有164頁\編于星期四\20點(diǎn)肉眼見腦體積和重量增加，腦回寬而扁平，腦溝狹窄，白質(zhì)水腫明顯，腦室縮小，腦疝形成。鏡下，腦組織疏松，細(xì)胞和血管周圍空隙變大，白質(zhì)中的變化較灰質(zhì)更加明顯。當(dāng)前第39頁\共有164頁\編于星期四\20點(diǎn)三、腦積水腦脊液量增多伴腦室擴(kuò)張稱為腦積水（hydrocephalus）。(thetotalCSFisabout140~150ml)腦積水的主要原因是腦脊液循環(huán)的通路被阻斷。onlyrarelydoesoverproductionofCSFbychoroidplexuspapillomas。腦室擴(kuò)張，腦組織萎縮。

當(dāng)前第40頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第41頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第42頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第43頁\共有164頁\編于星期四\20點(diǎn)1當(dāng)前第44頁\共有164頁\編于星期四\20點(diǎn)SECTION3

ACUTEPYOGENIC(BACTERIAL)MENINGITIS

急性化膿性腦脊髓膜炎

/流行性腦脊髓膜炎

當(dāng)前第45頁\共有164頁\編于星期四\20點(diǎn)1.INTRODUCTIONAcutepyogenicmeningitisreferstoaninflammatoryprocessoftheleptomeningesandCSFwithinthesubarachnoidspacewithpleocytosis軟腦膜化膿性炎癥當(dāng)前第46頁\共有164頁\編于星期四\20點(diǎn)ETIOLOGYANDPATHOGENESIS

病因和發(fā)病機(jī)制

流行性腦脊髓膜炎:腦膜炎雙球菌

當(dāng)前第47頁\共有164頁\編于星期四\20點(diǎn)2.1Causativeorganisms化膿性腦脊髓膜炎Varywiththeageofthepatient:Inneonates:Escherichiacoli,groupBstreptococciIninfantsandchildren:HemphilusinfluenzaeInadolescentsandyoungadults:NeisseriameningitidisIntheelderly:Streptococcuspneumoniae,Neisseriameningitidis(menigococcus)當(dāng)前第48頁\共有164頁\編于星期四\20點(diǎn)2.2PathogeneticmechanismBacteria→spreadviathesputum(sneeze)→intotherespiratorysystem→blood(bacteremia,septicemia)→meningitis

經(jīng)呼吸道傳染,引起的軟腦膜化膿性炎癥。當(dāng)前第49頁\共有164頁\編于星期四\20點(diǎn)MORPHOLOGY

病理變化

Grossly:---cloudyandsometimesfranklypurulentCSF---purulentexudatewithintheleptomeningesoverthesurfaceofthebrain---engorgedmeningealvessels

---腦脊膜血管充血，蛛網(wǎng)膜下腔充滿膿性滲出物，覆蓋腦溝腦回，可引起不同程度的腦室擴(kuò)張當(dāng)前第50頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第51頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第52頁\共有164頁\編于星期四\20點(diǎn)3.MORPHOLOGY(continued)Microscopically:

---hyperemiaintheleptomeninges---neutrophilsfilltheentiresubarachnoidspaceorarefoundpredominantlyaroundtheleptomeningealbloodvessels---蛛網(wǎng)膜血管擴(kuò)張充血，蛛網(wǎng)膜下腔有大量中性粒細(xì)胞及纖維蛋白滲出和少量單核細(xì)胞、淋巴細(xì)胞浸潤當(dāng)前第53頁\共有164頁\編于星期四\20點(diǎn)急性化膿性腦膜炎當(dāng)前第54頁\共有164頁\編于星期四\20點(diǎn)CLINICALCOURSE

臨床病理聯(lián)系

4.1Generalsignsofinfection感染性全身性癥狀:---highfever,hepatolienomegaly,petechialhemorrhage(skinrash)

神經(jīng)系統(tǒng)癥狀表現(xiàn):4.2Symptomsofincreasedintracranialpressure---顱內(nèi)壓升高癥狀：severeheadache,projectilevomiting4.3Signsofmeningealirritation:---腦膜刺激癥狀：photophobia,irritability,cloudingofconsciousness,episthotonus

andneckstiffness當(dāng)前第55頁\共有164頁\編于星期四\20點(diǎn)Episthotonus角弓反張當(dāng)前第56頁\共有164頁\編于星期四\20點(diǎn)CLINICALCOURSE4.4CranialnervesIIIthroughVIIparalysis顱神經(jīng)麻痹4.5Spinaltap腦脊液檢查是本病診斷的一個(gè)重要依據(jù):---cloudyorfranklypurulentCSF---increasedneutrophils---raisedproteinlevel---reducedglucosecontent---bacteriamaybeseenorcultured當(dāng)前第57頁\共有164頁\編于星期四\20點(diǎn)5.OUTCOMESANDPROGNOSIS結(jié)局和并發(fā)癥

5.1Recoveryingeneral多可痊愈5.2Chronicmeningitisandsequela后遺癥:①腦積水；②顱神經(jīng)受損麻痹；③腦底脈管炎致管腔阻塞，引起相應(yīng)部位腦缺血和梗死---leptomeningealfibrosis---hydrocephalus---cerebralinfarction---cranialnervesparalysis5.3Afewcasesdieoffulminantmeningitis暴發(fā)性腦膜炎:---septicemia,DIC,shock敗血癥、彌漫性血管內(nèi)凝血、休克---commonlyinchildren---pooroutcome病情兇險(xiǎn)，一般在起病24小時(shí)內(nèi)死亡當(dāng)前第58頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第59頁\共有164頁\編于星期四\20點(diǎn)SECTION4

EPIDEMICENCEPHALITISB(VIRALENCEPHALITISB)

流行性乙型腦炎當(dāng)前第60頁\共有164頁\編于星期四\20點(diǎn)1.DEFINITIONAparenchymalviralinfectionofthebraincausesneuronalandglialdamagewithassociatedinflammationandedema.Itisalmostinvariablyassociatedwithmeningealinflammation乙型腦炎病毒感染所致的急性傳染病Thehistologicfeatures:---perivascularandparenchymalmononuclearcellinfiltrates---glialnodules---neuronophagia---viralinclusionbodies當(dāng)前第61頁\共有164頁\編于星期四\20點(diǎn)2.ETIOLOGYANDPATHOGENESIS病因及傳染途徑

EncephalitisBvirus(arbovirus)→byinoculationthroughtheskinviathemosquitobites→enterthebody→blood(viremia)→brain乙型腦炎病毒為RNA病毒，其傳播媒介為蚊和長期貯存宿主當(dāng)前第62頁\共有164頁\編于星期四\20點(diǎn)3.MORPHOLOGY病理變化

Grossly:病變部位：中樞神經(jīng)系統(tǒng)灰質(zhì)，以大腦皮質(zhì)及基底核、視丘最為嚴(yán)重

---hyperemiainmeninges,cerebraledema,brainnecrosis

腦膜充血，腦水腫明顯，切面見粟粒大小的軟化灶

Microscopically:---perivascularinflammatorycells血管變化和炎癥反應(yīng)，

圍管狀浸潤

---manyfocalareasofnecrosis軟化灶形成---selectiveneuronaldegenerationandnecrosiswithneuronophagia、formationofsatellitosis神經(jīng)細(xì)胞變性、壞死

出現(xiàn)衛(wèi)星現(xiàn)象，噬神經(jīng)細(xì)胞現(xiàn)象---glialnodules膠質(zhì)細(xì)胞增生、膠質(zhì)細(xì)胞結(jié)節(jié)、膠質(zhì)瘢痕

當(dāng)前第63頁\共有164頁\編于星期四\20點(diǎn)乙腦，淋巴細(xì)胞套400倍當(dāng)前第64頁\共有164頁\編于星期四\20點(diǎn)乙腦，篩狀軟化灶200倍當(dāng)前第65頁\共有164頁\編于星期四\20點(diǎn)噬神經(jīng)細(xì)胞現(xiàn)象當(dāng)前第66頁\共有164頁\編于星期四\20點(diǎn)乙腦，膠質(zhì)細(xì)胞結(jié)節(jié)400倍當(dāng)前第67頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第68頁\共有164頁\編于星期四\20點(diǎn)4.CLINICALCOURSE

臨床病理聯(lián)系

4.1Generalizedneurologicdeficits:---seizures,confusion,delirium,stupororcoma嗜睡、昏迷4.2Focalsigns:---reflexasymmetry,ocularpalsies顱神經(jīng)麻痹癥狀4.3CSF腦脊液中細(xì)胞數(shù)增多:---colorless,increasedlymphocytes,raisedproteinlevel,normalsugarcontent當(dāng)前第69頁\共有164頁\編于星期四\20點(diǎn)CLINICALCOURSE4.4Symptomsofincreasedintracranialpressure腦水腫和顱內(nèi)壓升高，可引起腦疝:---headache,projectilevomiting,herniation4.5Signsofmeningealirritation腦膜刺激癥狀:

---neckstiffness當(dāng)前第70頁\共有164頁\編于星期四\20點(diǎn)5.OUTCOMES5.1Recoveryingeneral:theillnesslastsforsomeweeks.5.2Sequela:---palsy,idiocy,dysphasia當(dāng)前第71頁\共有164頁\編于星期四\20點(diǎn)SECTION5

DEGENERATIVEDISEASES神經(jīng)系統(tǒng)變性疾病

Aheterogeneousgroupofdisorderscharacterizedbyspontaneous,progressivedegenerationofneuronsinaspecificregionofsysteminthebrain,spinalcord,orboth

一組原因不明的中樞神經(jīng)系統(tǒng)疾病，為選擇性地累及某1-2個(gè)功能系統(tǒng)神經(jīng)細(xì)胞，病變特點(diǎn)：受累神經(jīng)元萎縮、死亡、星形膠質(zhì)細(xì)胞增生。Puredementiaanddementiaplussyndromes(e.g.ADandPDwithdementia)

累及大腦皮層→癡呆，累及基底核錐體外系→運(yùn)動(dòng)障礙，累及小腦→共濟(jì)失調(diào)Sporadicorfamilial當(dāng)前第72頁\共有164頁\編于星期四\20點(diǎn)（一）Alzheimer,sDisease(AD)

早老性癡呆/阿爾茨海默病Themostcommoncauseofdementiaintheelder(~60%)MostcasesofADoccuraftertheageof50起病在50歲以后，發(fā)病率呈增高趨勢(shì)

Progressiveimpairmentofmemoryandothercognitivefunctions以進(jìn)行性癡呆為主要臨床表現(xiàn)的大腦變性性疾病當(dāng)前第73頁\共有164頁\編于星期四\20點(diǎn)1.MORPHOLOGY病理變化

LOCATION:

Thecerebralcortex大腦皮層Grossly:---Corticalatrophy,narrowcerebralcorticalgyriandwideningsulci,ventricularsymmetricallyenlargement.腦萎縮明顯，腦回窄，腦溝寬；以頂、額、顳葉明顯。當(dāng)前第74頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第75頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第76頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第77頁\共有164頁\編于星期四\20點(diǎn)1.MORPHOLOGYMicroscopically:---Neurofibrillarytangles:anintracellularlesion,pairedhelicalstrandsoftauproteinclosetonucleiofneurons.Mainlyaffectingpyramidalcellsofcortex

神經(jīng)原纖維纏結(jié)：神經(jīng)細(xì)胞內(nèi)神經(jīng)原纖維增粗扭曲形成纏結(jié)，電鏡下為雙螺旋纏繞的細(xì)絲構(gòu)成；為神經(jīng)元趨向死亡標(biāo)志，多見于不同部位的錐體細(xì)胞。

當(dāng)前第78頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第79頁\共有164頁\編于星期四\20點(diǎn)1.MORPHOLOGYMicroscopically:---Senileplaques:acomplexextracellularlesionof15~100micon(um),aggregatesoffilamentswithacentralamyloidcoreinthecerebralcortex

老年斑：細(xì)胞外結(jié)構(gòu)，直徑20-150μm斑塊中心為一均勻的嗜銀團(tuán)，中心周圍有空暈，外圍有不規(guī)則嗜銀顆?；騾矤钗镔|(zhì)，電鏡下：由多個(gè)異常擴(kuò)張變性之軸索突觸終末構(gòu)成

當(dāng)前第80頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第81頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第82頁\共有164頁\編于星期四\20點(diǎn)1.MORPHOLOGYMicroscopically:---Amyloidangiopathy---Granulovacuolardegeneration神經(jīng)細(xì)胞內(nèi)顆?？张葑冃?--Hiranobodies神經(jīng)細(xì)胞樹突近端棒形嗜酸性包含體，生化證實(shí)為肌動(dòng)蛋白當(dāng)前第83頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第84頁\共有164頁\編于星期四\20點(diǎn)2.CAUSATION病因ThecauseofADremainsunknown病因及發(fā)病機(jī)制：不明

---themostcommonaremutationsinthepresenilin-1gene(onchromosome14)(早衰蛋白).

---expressionofspecificallelesofapoproteinE.---depositionofamyloid.可能有關(guān)因素：（1）受教育程度（2）遺傳因素，與第21對(duì)染色體等有關(guān)（與Down綜合癥基因接近）（3）神經(jīng)細(xì)胞代謝改變（4）繼發(fā)性的遞質(zhì)變化

當(dāng)前第85頁\共有164頁\編于星期四\20點(diǎn)3.DIAGNOSIS診斷

thesimplepresenceofplaquesand/ortanglesisnotspecificforAD.thenumberofplaquesandtanglesinneocorticalareasinthesettingofdementiaallowsonetomakeadiagnosisofAD,mainlyforresearchpurposes.diagnosismaybeestablishedduringlifebyearlymemoryfailure,slowprogressionandexclusionofothercauses.必須根據(jù)老年斑和神經(jīng)原纖維纏結(jié)的數(shù)目及部位并結(jié)合年齡及臨床表現(xiàn)作出診斷當(dāng)前第86頁\共有164頁\編于星期四\20點(diǎn)阿爾茨海默病美國診斷標(biāo)準(zhǔn)Patientageplaques（×200，/mm2）tangles（×200，/mm2）<50歲

>2~5

>2~5

≤60

>8

>2~5

≤75

>10

>2~5

>75

>10

當(dāng)前第87頁\共有164頁\編于星期四\20點(diǎn)4.PROGNOSIS

Deathusuallyresultsfromintercurrentbronchopneumoniaorotherinfections.5~6yearssurvivallengthafterdiagnosis.5-6年內(nèi)死于繼發(fā)感染，全身衰竭

當(dāng)前第88頁\共有164頁\編于星期四\20點(diǎn)

（二）Parkinson,sDisease

帕金森病/震顫性麻痹

Adegenerativedisorderinvolvingthedopamine-secretingneuronsofthesubstantianigraandthelocusceruleus與紋狀體、黑質(zhì)多巴胺神經(jīng)元變性有關(guān)

Mostcasesbecomemanifestbythesixthdecade,usuallysporadic.M:F=3:2是一種緩慢進(jìn)行性疾病，50-80歲多見

Clinically,rigidity,expressionlessfacies,stoopedposture,gaitdisturbances,slowingofvoluntarymovements,andacharacteristic“pill-rolling”tremor臨床有震顫，肌強(qiáng)直，步態(tài)不穩(wěn)，運(yùn)動(dòng)困難，假面具樣面容

當(dāng)前第89頁\共有164頁\編于星期四\20點(diǎn)1.MORPHOLOGYLOCATION:Thebrainstem,thebasalnucleusGrossly:---externallynormalormildlyatrophicbrain.---Pallorofthesubstantianigra(containspigmentedcells---“black”appearance)andlocusceruleus中腦黑質(zhì)和藍(lán)斑脫色為特征性肉眼變化

當(dāng)前第90頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第91頁\共有164頁\編于星期四\20點(diǎn)1.MORPHOLOGYMicroscopically:---Lossofthepigmented,catecholaminergicneurons.Remainingneuronscontainatypicaleosinophilicinclusionsinthecytoplasm---lewybodies

為該處神經(jīng)黑色素細(xì)胞喪失。Lewy小體形成：胞漿內(nèi)圓形，中心嗜酸性折光，邊緣色淺；電鏡下顯示由細(xì)絲構(gòu)成

---theneuropilisglioticandcontainsscatteredaccumulationsofneuromelaninpigment.當(dāng)前第92頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第93頁\共有164頁\編于星期四\20點(diǎn)2.ETIOLOGYANDPATHOGENESISThecause(s)ofPDisunknown病因及發(fā)病機(jī)制：不明Adisturbanceinthedopaminergicpathwaysconnectingthesubstantianigratothebasalganglia.Adisturbanceinmotorfunctions.當(dāng)前第94頁\共有164頁\編于星期四\20點(diǎn)3.DIAGNOSISANDPROGNOSISThediagnosisintheearlystageisdifficult.Deathusuallyresultsfromintercurrentinfectionortraumafromfrequentfallscausedbyposturalinstability.Thecourseofthediseaseisoneofsteadyprogression,usuallyoveraperiodofabout10years.Treatmentissymptomaticanddoesnothaltthepathologicalprocess.抗膽堿及多巴胺前體藥作用有效

當(dāng)前第95頁\共有164頁\編于星期四\20點(diǎn)3當(dāng)前第96頁\共有164頁\編于星期四\20點(diǎn)SECTION6

TUMORS神經(jīng)系統(tǒng)腫瘤

當(dāng)前第97頁\共有164頁\編于星期四\20點(diǎn)1.INTRODUCTION1.1TheincidenceoftumorsoftheCNS:---10~17per100,000personsforintracranialtumors---1~2per100,000personsforintraspinaltumors---abouthalfareprimarytumorsandtherestaremetastatic當(dāng)前第98頁\共有164頁\編于星期四\20點(diǎn)INTRODUCTION1.2Location:---inchildhood:70%ofprimarytumorsariseintheposteriorfossa---inadults:acorrespondingproportionariseabovethetentorium當(dāng)前第99頁\共有164頁\編于星期四\20點(diǎn)INTRODUCTION1.3Classification

1.3.1TumorsoftheCNS:------gliomas(40%),meningiomas(15%),neuronaltumors,medulloblastoma1.3.2Tumorsoftheperipheralnerve:------schwannoma(5%),neurofibroma1.3.3Metastatictumor當(dāng)前第100頁\共有164頁\編于星期四\20點(diǎn)INTRODUCTION1.4intracranialtumorsymptoms1.Localnervoussymptoms:

palsy癱瘓，epilepsy癲癎2.Increasedintracranialpressure:headache,projectilevomiting當(dāng)前第101頁\共有164頁\編于星期四\20點(diǎn)2.COMMONTUMORS2.1Gliomas膠質(zhì)瘤2.1.1Tumortypes:Astrocytomas星形膠質(zhì)細(xì)胞瘤:30%ofintracranialtumors,>78%ofgliomasOligodendrogliomas少突膠質(zhì)細(xì)胞瘤:about5~10%ofgliomasEpendymomas室管膜（細(xì)胞）瘤:5~10%ofgliomas當(dāng)前第102頁\共有164頁\編于星期四\20點(diǎn)COMMONTUMORS2.1.2Uniquecharacteristics不同于其它部位的腫瘤其生物學(xué)特性:

---thedistinctionbetweenbenignandmalignantlesionsislessevident(lackcapsule)thaninothersites良惡性的相對(duì)性，無包膜，均呈浸潤性生長，間變的界限反而清楚---theabilitytosurgicallyremovetheneoplasmisrestrictedbyfunctionalanatomicconsiderations---theprimaryCNSneoplasmsspreadbythecerebrospinalfluid(CSF)

.Itis

veryraretometastasizeoutsideoftheCNS轉(zhuǎn)移：①腦脊液轉(zhuǎn)移為常見方式②顱外轉(zhuǎn)移極少見當(dāng)前第103頁\共有164頁\編于星期四\20點(diǎn)2.1.3Astrocytoma星形膠質(zhì)細(xì)胞瘤

80%ofadultprimarybraintumorsLocation:cerebralhemispherescerebellumbrainstemspinalcord當(dāng)前第104頁\共有164頁\編于星期四\20點(diǎn)AstrocytomaMorphology(grossly):腫瘤為結(jié)節(jié)狀、巨塊狀，瘤體灰白色，可呈膠凍狀外觀，形成大小不等的囊腔。---lowgrade:poorlydefined,gray-white,infiltrativetumors分化較好的腫瘤，境界不清---highgrade(glioblastomamultiforme):amixtureoffirm,whiteareasandsofter,yellowfociofnecrosisaswellascysticchangeandhemorrhage分化程度較低的腫瘤則境界分明當(dāng)前第105頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第106頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第107頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第108頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第109頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第110頁\共有164頁\編于星期四\20點(diǎn)Astrocytoma星形膠質(zhì)細(xì)胞瘤Microscopically分纖維型、原漿型和肥胖型，高度惡性的星形膠質(zhì)細(xì)胞瘤稱為多形性膠質(zhì)母細(xì)胞瘤（glioblastomamultiforme）。膠質(zhì)纖維酸性蛋白（GFAP）呈陽性反應(yīng)，是該腫瘤的特異標(biāo)志。

---theunifyinghistologicfeature:

---thebackgroundoftheneoplasticastrocyticnuclei,hypercellularityandsomenuclearpleomorphism,mitosesandvascularproliferation---thepresenceofnecrosisisthedefininghistologicfeatureofglioblastomamultiforme當(dāng)前第111頁\共有164頁\編于星期四\20點(diǎn)纖維型星形細(xì)胞瘤當(dāng)前第112頁\共有164頁\編于星期四\20點(diǎn)纖維型星形細(xì)胞瘤當(dāng)前第113頁\共有164頁\編于星期四\20點(diǎn)原漿型星形細(xì)胞瘤當(dāng)前第114頁\共有164頁\編于星期四\20點(diǎn)惡性原漿型星形細(xì)胞瘤當(dāng)前第115頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第116頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第117頁\共有164頁\編于星期四\20點(diǎn)AstrocytomaPrognosis:---verypoor,themeanlengthofsurvivalafterdiagnosisisonly8~10months---butpilocyticastrocytomasarealmostinvariablybenignbehavioramongthegliomas當(dāng)前第118頁\共有164頁\編于星期四\20點(diǎn)2.1.4Oligodendroglioma

少突膠質(zhì)細(xì)胞瘤

constituteabout5~10%ofgliomasLocation:cerebralhemispheres好發(fā)于大腦皮質(zhì)的淺層，尤以左額葉為多見當(dāng)前第119頁\共有164頁\編于星期四\20點(diǎn)OligodendrogliomaGrossly:腫瘤呈灰紅色、邊界清楚的球形腫塊，可發(fā)生囊性變、出血和鈣化，其中鈣化灶對(duì)X線診斷有一定幫助---well-circumscribed,gelatinous,graymasses---oftenwithcysts,focalhemorrhage,calcificationMicroscopically:瘤細(xì)胞大小均勻，形態(tài)單一，彌漫排列，但有環(huán)繞神經(jīng)元呈衛(wèi)星狀排列的傾向---sheetsofregularcellswithsphericalnucleisurroundedbyaclearhaloofcytoplasmPrognosis:betterprognosisthanastrocytomas,10-30yearssurvivallength.當(dāng)前第120頁\共有164頁\編于星期四\20點(diǎn)少突膠質(zhì)細(xì)胞瘤當(dāng)前第121頁\共有164頁\編于星期四\20點(diǎn)2.1.5Ependymoma室管膜瘤

Location:oftenarisenexttotheependymallinedventricularsystem起源于室管膜細(xì)胞，可發(fā)生于腦室系統(tǒng)任何部位，以第四腦室最多見

---inthefirsttwodecadesgroup:nearthefourthventricle,whichconstitute5~10%oftheprimarybraintumors---inmiddlelife:inthespinalcordincludingtheoft-obliteratedcentralcanalofthespinalcord當(dāng)前第122頁\共有164頁\編于星期四\20點(diǎn)EpendymomaMorphologyGrossly:---inthefourthventricle:solidorpapillarymasses---intheintraspinalcord:masswithsharpdemarcation當(dāng)前第123頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第124頁\共有164頁\編于星期四\20點(diǎn)EpendymomaMorphologyMicroscopically:---cellswithregular,roundtoovalnucleiwithabundantgranularchromatinwithafinefibrillarybackground---tumorcellsmayformgland-likestructureswithlong,delicateprocessesextendingintoalumen(ependymalrosettes,morefrequentlyperivascularpseudorosettes)(thatresembletheembryologicependymalcanal)當(dāng)前第125頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第126頁\共有164頁\編于星期四\20點(diǎn)乳頭狀型當(dāng)前第127頁\共有164頁\編于星期四\20點(diǎn)EpendymomaPrognosis:Hydrocephalus:secondarytoprogressiveobstructionCSFdisseminationoftumorAnaveragesurvivalofabout4yearsfollowingtreatment(poorprognosis)當(dāng)前第128頁\共有164頁\編于星期四\20點(diǎn)THESPECTRUMOFGLIOMASCLINICALTERMLOCATIONMAJORPATHOLOGICCHANGEPROGNOSISAstrocy-tomaBrain~spinalcordPoorlydefined,gray-white,infiltrativetumors,yellowfociofnecrosis,cysticchangeandhemorrhage.Neoplasticastrocyticnuclearpleomorphism,hypercellularity,mitoses,necrosis,vascularproliferationverypoor,8~10monthssurvivallengthOligo-dendro-gliomaCerebralhemispheresWell-circumscribed,gelatinous,graymasses,withcysts,focalhemorrhage,calcification.Sheetsofregularcells,sphericalnucleisurroundedbyaclearhaloofcytoplasmbetterprognosis,10~30yearssurvivallengthEpendy-momaVentricularsystemSolid,papillarymasses(4thventricle),masswithsharpdemarcation(intraspinalcord).Cellswithregular,roundtoovalnucleiwithafinefibrillarybackground

mayformependymalrosettes,pseudorosettesabout4yearssurvivallength當(dāng)前第129頁\共有164頁\編于星期四\20點(diǎn)2.2Medulloblastoma髓母細(xì)胞瘤

Patients:---predominantlyinchildren中樞神經(jīng)系統(tǒng)常見的原始神經(jīng)上皮腫瘤。好發(fā)于兒童Location:---exclusivelyinthemidlineofthecerebelluminchildren---laterallocationsinadults多發(fā)于小腦蚓部，在成人則多見于小腦半球

當(dāng)前第130頁\共有164頁\編于星期四\20點(diǎn)MedulloblastomaMorphologyGrossly:---wellcircumscribed,gray,andfriabletumor

瘤組織呈魚肉狀，色灰紅Microscopically:---usuallyextremelycellular,withsheetsofanaplasticcells(highlymalignant)---hyperchromatic,roundorelongatednuclei,abundantmitoses,littlecytoplasm

腫瘤細(xì)胞核深，核分裂像，胞漿少而邊界不清，細(xì)胞密集。典型的菊形團(tuán)，對(duì)髓母細(xì)胞瘤的病理診斷有意義當(dāng)前第131頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第132頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第133頁\共有164頁\編于星期四\20點(diǎn)MedulloblastomaComplicationExtensionintothesubarachnoidspaceDisseminationthroughtheCSF("drop"metastases)當(dāng)前第134頁\共有164頁\編于星期四\20點(diǎn)MedulloblastomaPrognosisTheprognosisforuntreatedpatientsisdismal.Itisexquisitelyradiosensitive.Withtotalexcisionandradiation,the5-yearsurvivalratehasbeenreportedtobeashighas75%當(dāng)前第135頁\共有164頁\編于星期四\20點(diǎn)2.3Meningiomas腦膜瘤

Theyarisefromtheeningothelialcellofthearachnoid多來源于埋在上矢狀竇兩側(cè)的蛛網(wǎng)膜絨毛的細(xì)胞巢Predominantlybenigntumorsofadults(M:F=2:3)本瘤大多為良性當(dāng)前第136頁\共有164頁\編于星期四\20點(diǎn)MeningiomasLocation:常見于上矢狀竇旁大腦鐮兩側(cè)，蝶骨嵴，嗅溝，小腦腦橋角---alonganyoftheexternalsurfacesofthebrainaswellaswithintheventricularsystem,wheretheyarisefromthestromalarachnoidcellsofthechoroidplexus---commonsites:theparasagittalaspectoftheconvexity,duraoverthelateralconvexity,thewingofthesphenoid,olfactorygrooveandtheforamenmagnum當(dāng)前第137頁\共有164頁\編于星期四\20點(diǎn)MeningiomasMorphologyGrossly:

---solitary,roundedmasseswithawell-definedduralbasethatcompressunderlyingbrain,usuallyencapsulatedwiththin,fibroustissue

---anothergrowthpattern:thetumorspreadsinasheet-likefashionalongthesurfaceofthedura---thelesionsareusuallyfirmtofibrous---lackevidenceofnecrosisorextensivehemorrhage腫瘤呈球形，分葉狀或不規(guī)則形，質(zhì)實(shí)或硬，邊界清楚有包膜，切面灰白色，呈顆粒狀、條索旋渦狀，有的質(zhì)地似砂礫樣當(dāng)前第138頁\共有164頁\編于星期四\20點(diǎn)腦膜瘤當(dāng)前第139頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第140頁\共有164頁\編于星期四\20點(diǎn)MeningiomasMorphologyMicroscopically:腦膜細(xì)胞型（融合細(xì)胞型），纖維細(xì)胞型，過度（混合）型(littleprognosticsignificance)

include:---syncytial:whorledclustersofcellsintightwithoutvisiblecellmembranes---fibroblastic:elongatedcellswithabundantcollagendeposition---transitional:sharesfeaturesofthesyncytialandfibroblastic當(dāng)前第141頁\共有164頁\編于星期四\20點(diǎn)合體細(xì)胞型當(dāng)前第142頁\共有164頁\編于星期四\20點(diǎn)纖維細(xì)胞型當(dāng)前第143頁\共有164頁\編于星期四\20點(diǎn)MeningiomasMorphology---psammomatous:withpsammomabodies,calcification---secretory:intracytoplasmicdropletsandintracellularlumina---microcystic:loose,spongyappearance---papillaryvariant:pleomorphiccellsarrangedaroundfibrovascularcores---angioblasticmeningioma當(dāng)前第144頁\共有164頁\編于星期四\20點(diǎn)纖維型過渡細(xì)胞型砂粒體瘤型血管瘤型當(dāng)前第145頁\共有164頁\編于星期四\20點(diǎn)MeningiomasMorphologyMalignantmeningiomas:---extremelyunusualtumorsandmaybedifficulttorecognizehistologically---featuresthatsupportthisdiagnosisinclude:singlecellinfiltrationofunderlyingbrainandabundantmitoseswithatypicalforms當(dāng)前第146頁\共有164頁\編于星期四\20點(diǎn)MeningiomasPrognosisSlow-growingCompressionofunderlyingbrain當(dāng)前第147頁\共有164頁\編于星期四\20點(diǎn)2.4Schwannoma神經(jīng)鞘瘤

Reviews:ThetransitionzonebetweencentralmyelinationbyoligodendrocytesandperipheralmyelinationbySchwanncellsoccurswithinseveralmillimetersoftheexitofaxonsfromthesubstanceofthebrain當(dāng)前第148頁\共有164頁\編于星期四\20點(diǎn)SchwannomaForcranialnervesIIIthroughXIIaswellasforallspinalroots,tumorderivedfromSchwanncellsandotherperineuralelementscanariseinlocationsthatcausepathologicchangesinadjacentbrainorspinalcord當(dāng)前第149頁\共有164頁\編于星期四\20點(diǎn)SchwannomaDefinition:---thebenigntumorsarisefromtheneuralcrest-derivedSchwanncellLocation:---mostcommoninthecerebellopontineangle(vestibularschwannomaor"acousticneuroma")---othercranialnerves(likesensorynerves)intherareinstances

可發(fā)生在周圍神經(jīng)、顱神經(jīng)或交感神經(jīng)當(dāng)前第150頁\共有164頁\編于星期四\20點(diǎn)MorphologyGrossly:---well-circumscribed,encapsulatedmassesthatareattachedtothenervebutcanbeseparatedfromit---tumorsarefirm,graymassesbutmayalsohaveareasofcysticchangeandayellow,xanthomatousappearance

神經(jīng)鞘瘤有完整的包膜，質(zhì)實(shí)，呈圓形或結(jié)節(jié)狀，常壓迫鄰近組織，與其發(fā)生的神經(jīng)粘連在一起。切面為灰白或灰黃色略透明，可見旋渦狀結(jié)構(gòu)當(dāng)前第151頁\共有164頁\編于星期四\20點(diǎn)當(dāng)前第152頁\共有164頁\編于星期四\20點(diǎn)MorphologyMicroscopicallyamixture