脫髓鞘病(7年制)課件

中樞神經(jīng)系統(tǒng)脫髓鞘疾病

DemyelinatingDiseasesoftheCentral

NervousSystem

中樞神經(jīng)系統(tǒng)脫髓鞘疾病

DemyelinatingDise1掌握MS概念、病因、發(fā)病機(jī)制、臨床表現(xiàn)、輔助檢查、治療、診斷標(biāo)準(zhǔn)及鑒別診斷。熟悉視神經(jīng)脊髓炎概念、臨床表現(xiàn)、輔助檢查、診斷及治療。了解MS病理、預(yù)后；急性播散性腦脊髓炎概念、臨床表現(xiàn)、診斷及治療。Keypoints-DemyelinatingDiseasesofCNS掌握MS概念、病因、發(fā)病機(jī)制、臨床表現(xiàn)、輔助檢查、治療、診斷2脫髓鞘病(7年制)課件3脫髓鞘病(7年制)課件4脫髓鞘病(7年制)課件5脫髓鞘病(7年制)課件62.PathologicFindings

①

DestructionofthemyelinsheathsofCNS;oftenprimarilyinwhitematter,eitherinmultiplesmalldisseminatedfociorinlargerfoci;2.PathologicFindings

①Destr7②Infiltrationofinflammatorycellsinaperivenousdistribution;③Arelativeintegrityoftheaxiscylindersinthelesionsandalackofwallerian,thesecondarydegenerationoffibertracts.②Infiltrationofinflammato8臨床常見脫髓鞘疾病急性播散性腦脊髓炎(acutedisseminatedencephalomyelitis,ADEM)多發(fā)性硬化癥(multiplesclerosis,MS)亞型視神經(jīng)脊髓炎(Devicdiseases)急性出血性白質(zhì)腦病(acutehemorrhageleukoencephalitis,AHLE)臨床常見脫髓鞘疾病多發(fā)性硬化癥(multiplescler9

多發(fā)性硬化癥(MS)

多發(fā)性硬化癥(MS)10多發(fā)性硬化

MultipleSclerosis,MS

1.Concept:

MsisakindofautoimmunediseasescharacterizedbydemyelinationofCNS.Duetoitshighincidence,chronicityandtendencytoattackyoungadults,ithasbecomeoneofthemostimportantCNSdiseases.多發(fā)性硬化

MultipleSclerosis,MS

1.11Therearemultipleareasof

demyelinationwithintheCNS.Theepisodesofdemyelinationareseparatedintimeandplace,andclassicallythediseaserunsarelapsing-remittingcourse.(brainandspinalcord)Therearemultipleareasofde12是一種常見以中樞神經(jīng)系統(tǒng)炎性脫髓鞘為特征的自身免疫性疾病病灶部位及時(shí)間上的多發(fā)性多數(shù)均以反復(fù)多次發(fā)作與緩解的病程具有免疫易感性、年輕人多見是一種常見以中病灶部位及時(shí)間上的多發(fā)性多數(shù)均以反復(fù)多次發(fā)具有132.EtiologyAndPathogenesis1)病毒感染及自身免疫反應(yīng)：Sincetheexactcauseisuncertain.Immunologicalmechanismsundoubtedlyplayarole,althoughthecausationisprobablymultifactorial.麻疹病毒,人類噬T淋巴細(xì)胞病毒(HTLV-I),分子模擬,細(xì)胞免疫及體液免疫。2)遺傳因素(inheritedfactor)3)環(huán)境因素(environment)2.EtiologyAndPathogenesis1)14脫髓鞘病(7年制)課件15脫髓鞘病(7年制)課件163.EpidemiologyIncidenceofMSassociatedwithlatitude.Onmovingfromahigh-prevalenceareatoalow-prevalenceareapriortopuberty,theriskofdevelopingMSishigherthaninthelow-prevalencearea;Howeverthemoveismadefollowingpuberty,theriskofthehigh-prevalenceisretained.3.EpidemiologyIncidenceofM17Hereditymaybeanimportantfactor.MSassociatedwiththeHLA-DRlocusonthesixthchromosome,HLA-DR2expressstronglyandthen-DR3,

B7andA3.Hereditymaybeanimporta18脫髓鞘病(7年制)課件194.

PathologicFindings

Characteristic:Multipledemyelinatedplaques.Position:Whitematteraroundthelateralventriclesandspinalcord,opticnerve,brainstemandcerebellar.

Acutestage:hyperemia,ondema,demyelination,

infiltriationofinflammatorycellsdistributedinperivenous.Recoverystage:Astrocyteproliferition,formingofastrocyticscab.

4.PathologicFindings

Charact20

急性期:充血、水腫、炎性脫髓鞘、血管周圍Lc浸潤(rùn)。

恢復(fù)期:星狀細(xì)胞增生、膠質(zhì)斑痕形成。

肉眼觀：CNS內(nèi)脫髓鞘斑塊急性期:充血、水腫、炎性脫髓鞘、血管周圍Lc浸215.ClinicalManifestations1)

Prodrome:Thesymptomsevolvedmoreslowly,overseveralweeksormonths.2)Acuteorsubacuteonset(Relapsing-remitting).5.ClinicalManifestations1)223)

Earlysymptomsandsigns:

①Weaknessornumbness;(1/2patientshaveparesthesiaononeormorelimbs)

②

Thevisuallossinoneorbotheyes;③Nystagmus;3)Earlysymptomsandsigns:

234)Commonsymptomsandsigns:①paralysisandparaplegia;②

Thevisuallossinoneorbotheyes;

(1/2patientshavevisualdisorders,relapsing-remitting)

③Nystagmusandpalsyofeyemuscles;(internuclearophthalmoplegia,PPRF

oneandahalfsyndrome)4)Commonsymptomsandsigns:①24“一個(gè)半綜合征”垂直眼震“一個(gè)半綜合征”垂直眼震25脫髓鞘病(7年制)課件26④Sensationdisorder:Romberg’ssign,

(>1/2)Lhermitte’ssign;⑤Ataxia(1/2),Charcot’ssyndrom(laterstage);⑥ImpairmentofPNS;⑦Attacksyndrom;⑧Otherclinicalfeature.④Sensationdisorder:Romberg’276.LaboratoryandassistantTests1)CSFTest

①NumberofMNC<15106/L;②IgG-Index>0.7(70%);oligoclonalbands(OB)(95%);

③MBP,PLP,MAG,MOGAbsandAb-secretingcells;

④CSF-Alb/serum-Alb>1.7(probabilityofMS)6.LaboratoryandassistantTe282)Evokedpotentials:50%-90%abnormal.

①visualevokedpotentials(VEP);②brainstemauditoryevokedpotentials(BAEP);③somatosensoryevokedpotentials(SEP).3)MRI:

①preiventricularplaques;②regularplaquesinbrainstem,cerebellumandspinalcord;③atrophysymptom.2)Evokedpotentials:50%-90%29脫髓鞘病(7年制)課件30脫髓鞘病(7年制)課件31脫髓鞘病(7年制)課件32脫髓鞘病(7年制)課件33脫髓鞘病(7年制)課件34-AbnormalMRIscansarefoundin

96%withadefinitediagnosisofMS

70%withadiagnosisofprobableMS

30-50%withadiagnosisofpossibleMSMRICriteriafordiagnosingMS

Atleast3Lesionsandtwoofthefollowing:1

LesionsabuttingtheLateralVentricles

2Lesionswithdiametersgreaterthan5mm3LesionspresentinthePosteriorFossaSource

(OffenbacherH,FazekasF,SchmidtRetal.

AssessmentOfMRICriteriaForADiagnosisOfMS*Neurology1993;43:905-909)-AbnormalMRIscansarefound35

Diagnosticcriteria1.ClinicaldefiniteMS(CDMS):

①twotimesofattackandtwolesions;②twoattacks,onelesionandonesubclinicalevidence;2.LaboratorysupporteddefiniteMS(LSDMS):

①Twoattacks,onesubclinicalevidenceandCSFOB/IgG;②Oneattack,twolesionsandCSFOB/IgG;③Oneattack,onelesion,onesubclinicalevidenceandCSFOB/IgG;

Diagnosticcriteria1.363.ClinicalprobableMS(CPMS):

①twoattacks,onelesion;②oneattack,twolesions;③oneattack,onelesionandothersubclinicalevidence;4.LaboratorysupportedprobableMS(LSPMS)

Twoattacks;CSFOB/IgG;TwoattacksinvolvingdifferentpartofCNS,intermissionatlestonemonth;eachattackmustcontinuefor24hs.3.ClinicalprobableMS(CPMS37多發(fā)硬化的診斷標(biāo)準(zhǔn)診斷發(fā)作次數(shù)臨床病灶數(shù)亞臨床證據(jù)CSFOB/IgG臨床確診（CDMS）2221及1實(shí)驗(yàn)室支持確診(LSDMS）211121或1及1+++臨床可能(CPMS)2111211+實(shí)驗(yàn)室支持可能(LSPMS）2兩次發(fā)作均累及CNS不同部位，間隔至少一個(gè)月，每次持續(xù)24小時(shí)。+多發(fā)硬化的診斷標(biāo)準(zhǔn)診斷發(fā)作次數(shù)臨床病灶數(shù)亞臨床證據(jù)CSFO38DifferentialDiagnosis1.急性播散性腦脊髓炎

2.腦動(dòng)脈炎、腦干炎、脊髓血管畸形3.頸椎病脊髓型

4.熱帶痙攣性截癱

5.大腦淋巴瘤DifferentialDiagnosis1.急性播散性39

Treatment目前尚無一種特效療法，治療的主要目的是：1.急性活動(dòng)期抑制其炎癥性脫髓鞘過程，遏止病情的進(jìn)展。

2.盡量預(yù)防能促發(fā)的外因，減少復(fù)發(fā)次數(shù)，延長(zhǎng)緩解間歇期。

3.預(yù)防并發(fā)癥。4.對(duì)癥及支持療法。

Treatment目前尚無一種特效療法，治療的主要目401.Relapsing-RemittingMS：

①

Anti-inflammatorytreatment:

methylprenisolone(highdosefor3d),prednison,dexamethasone;

②

Suppressionormodulationoftheimmunesystem:IFN-1

and1b

;

Azathioprine；

Immuneglublin(Ig):0.4g/kg.dIVIg3-5d

脫髓鞘病(7年制)課件412.ProgressiveMS：

①M(fèi)ethotrexate,MTX;

②Cyclosphoamide;③CyclosporineA;④Plasmatransplantation.

2.ProgressiveMS：

①M(fèi)423.

Symptomatictreatment:

①Spasticity:baclofen,dantrolene,diazepamandtizanidinecanbehelpful.

②Bladderdysfunction:anticholinergicdrugsurinarycathetermayberequired.3.Symptomatictreatment:

43預(yù)后分型1.良性型2.復(fù)發(fā)-緩解3.緩慢進(jìn)展型4.慢性進(jìn)展型預(yù)后分型1.良性型44Examples患者，女，32歲。主訴：行走不穩(wěn)1年，左耳鳴、視物雙影半年。走路不穩(wěn)，踩棉花感左耳鳴復(fù)視快速細(xì)小水平眼震向右凝視時(shí)明顯右側(cè)指鼻試驗(yàn)、輪替試驗(yàn)、跟膝脛試驗(yàn)均欠佳

Romberg征(+)，左Hoffmann征(+)四肢腱反射增高，以雙下肢腱反射增高，右側(cè)踝陣攣陽性頭顱MRI未見異常Examples患者，女，32歲。主訴：行走不穩(wěn)1年，左耳鳴45該患如何定位及定性？該患如何定位及定性？46

視神經(jīng)和脊髓受累較多見，病灶中的軟化、壞死較多見.視神經(jīng)脊髓炎又稱Devic病,為多發(fā)性硬化的一個(gè)亞型.中國(guó),日本等東方人1.Introduction2.EtiologyAndPathogenesis視神經(jīng)脊髓炎

(Neuromyelitisoptica,NMO)

中國(guó),日本1.Introduction2.Etiology473.Patholgy主要侵犯視神經(jīng)、視交叉、和脊髓胸頸段3.Patholgy主要侵犯視神經(jīng)、視交叉、和脊髓胸頸段48Acutestage:infiltrationofinflammatorycells.AstrocyteproliferitionAcutestage:infiltrationofAs49ClinicalManifestations-NMO1.年輕居多，21-41歲。2.特征：急性橫貫性脊髓炎和雙側(cè)同時(shí)或相繼出現(xiàn)的ON。70%可在數(shù)日內(nèi)有截癱。3.急性起病可在數(shù)小時(shí)或數(shù)日內(nèi)單或雙眼失明，眼眶痛。4.脊髓癥狀可橫貫、不對(duì)稱、或呈播散性；特征為快速進(jìn)展的雙下肢癱，感覺脫失平面、括約肌障礙等，1/3病人有Lhermitte征、根痛。ClinicalManifestations-NMO1.50一、輔助檢查1.CSF細(xì)胞數(shù)增加，73%單相、82%復(fù)發(fā)。2.復(fù)發(fā)病人脊髓MRI88%出現(xiàn)縱向融合超過數(shù)個(gè)節(jié)段，釓強(qiáng)化和腫脹常見。二、鑒別診斷1.單純球后神經(jīng)炎2.MS表現(xiàn)為NMO臨床模式。3.亞急性視神經(jīng)病三、治療大劑量甲強(qiáng)沖擊療法一、輔助檢查51

頸髓脫髓鞘視神經(jīng)炎

頸髓脫髓鞘視神經(jīng)炎52急性播散性腦脊髓炎

(acutedisseminatedencephalomylitis,ADEM)1.Introduction:ADEMisakindofacuteinflammatorydisseminateddiseaseinvolvedthewhitermatterofbrainandspinalcord.

(感染出疹或疫苗接種)爆發(fā)型：急性出血性白質(zhì)腦炎(acutenecrotizinghemorrhagicencephalomyelitis,AHL)急性播散性腦脊髓炎

(acutedisseminated532.EtiologyAndPathogenesis病毒感染，腦組織+FAC可誘發(fā)EAE，認(rèn)為ADEM是急性MS或其變異型。2.EtiologyAndPathogenesis543.Pathology

腦和脊髓多數(shù)脫髓鞘病灶,小靜脈周圍炎性反應(yīng)，形成血管袖套。

3.Pathology55脫髓鞘病(7年制)課件56

ClinicalManifestations1.Prodrome2.Typeofencephalitis3.Typeofmeningitis4.TypeofmyelitisClinicalManifestations1.Pr571.LabTeste1)WBC,pressureofCSFornormal,Pr,IgGandOB(+);2)AbnormalofEEG;3)CTscanshowsthelesionsofmultiplediffusionsubcortexlowdesity;MRIshowsthemultiplelesionsofabnormalT1,T2inbrainandwhitermatter.

1.LabTeste582.Diagnosisanddifferentialdiagnosis

乙腦及單皰病腦3.Treatment

大量皮質(zhì)類固醇沖擊療法2.Diagnosisanddifferen59Otherdemyelinatingdiseases

1.Diffusesclerosis2.Leukodystrophy3.Centralpontinemyelinolysis,CPM4.ConcentricsclerosisofBaloOtherdemyelinatingdiseases

1601.Diffusesclerosis(Schilder’s)

1.Diffusesclerosis(Schilder’612.

Leukodystrophy2.Leukodystrophy623.Centralpontinemyelinolysis,CPM

3.Centralpontinemyeli634.ConcentricsclerosisofBalo4.Concentricsclerosisof64謝謝謝謝65中樞神經(jīng)系統(tǒng)脫髓鞘疾病

DemyelinatingDiseasesoftheCentral

NervousSystem

中樞神經(jīng)系統(tǒng)脫髓鞘疾病

DemyelinatingDise66掌握MS概念、病因、發(fā)病機(jī)制、臨床表現(xiàn)、輔助檢查、治療、診斷標(biāo)準(zhǔn)及鑒別診斷。熟悉視神經(jīng)脊髓炎概念、臨床表現(xiàn)、輔助檢查、診斷及治療。了解MS病理、預(yù)后；急性播散性腦脊髓炎概念、臨床表現(xiàn)、診斷及治療。Keypoints-DemyelinatingDiseasesofCNS掌握MS概念、病因、發(fā)病機(jī)制、臨床表現(xiàn)、輔助檢查、治療、診斷67脫髓鞘病(7年制)課件68脫髓鞘病(7年制)課件69脫髓鞘病(7年制)課件70脫髓鞘病(7年制)課件712.PathologicFindings

①

DestructionofthemyelinsheathsofCNS;oftenprimarilyinwhitematter,eitherinmultiplesmalldisseminatedfociorinlargerfoci;2.PathologicFindings

①Destr72②Infiltrationofinflammatorycellsinaperivenousdistribution;③Arelativeintegrityoftheaxiscylindersinthelesionsandalackofwallerian,thesecondarydegenerationoffibertracts.②Infiltrationofinflammato73臨床常見脫髓鞘疾病急性播散性腦脊髓炎(acutedisseminatedencephalomyelitis,ADEM)多發(fā)性硬化癥(multiplesclerosis,MS)亞型視神經(jīng)脊髓炎(Devicdiseases)急性出血性白質(zhì)腦病(acutehemorrhageleukoencephalitis,AHLE)臨床常見脫髓鞘疾病多發(fā)性硬化癥(multiplescler74

多發(fā)性硬化癥(MS)

多發(fā)性硬化癥(MS)75多發(fā)性硬化

MultipleSclerosis,MS

1.Concept:

MsisakindofautoimmunediseasescharacterizedbydemyelinationofCNS.Duetoitshighincidence,chronicityandtendencytoattackyoungadults,ithasbecomeoneofthemostimportantCNSdiseases.多發(fā)性硬化

MultipleSclerosis,MS

1.76Therearemultipleareasof

demyelinationwithintheCNS.Theepisodesofdemyelinationareseparatedintimeandplace,andclassicallythediseaserunsarelapsing-remittingcourse.(brainandspinalcord)Therearemultipleareasofde77是一種常見以中樞神經(jīng)系統(tǒng)炎性脫髓鞘為特征的自身免疫性疾病病灶部位及時(shí)間上的多發(fā)性多數(shù)均以反復(fù)多次發(fā)作與緩解的病程具有免疫易感性、年輕人多見是一種常見以中病灶部位及時(shí)間上的多發(fā)性多數(shù)均以反復(fù)多次發(fā)具有782.EtiologyAndPathogenesis1)病毒感染及自身免疫反應(yīng)：Sincetheexactcauseisuncertain.Immunologicalmechanismsundoubtedlyplayarole,althoughthecausationisprobablymultifactorial.麻疹病毒,人類噬T淋巴細(xì)胞病毒(HTLV-I),分子模擬,細(xì)胞免疫及體液免疫。2)遺傳因素(inheritedfactor)3)環(huán)境因素(environment)2.EtiologyAndPathogenesis1)79脫髓鞘病(7年制)課件80脫髓鞘病(7年制)課件813.EpidemiologyIncidenceofMSassociatedwithlatitude.Onmovingfromahigh-prevalenceareatoalow-prevalenceareapriortopuberty,theriskofdevelopingMSishigherthaninthelow-prevalencearea;Howeverthemoveismadefollowingpuberty,theriskofthehigh-prevalenceisretained.3.EpidemiologyIncidenceofM82Hereditymaybeanimportantfactor.MSassociatedwiththeHLA-DRlocusonthesixthchromosome,HLA-DR2expressstronglyandthen-DR3,

B7andA3.Hereditymaybeanimporta83脫髓鞘病(7年制)課件844.

PathologicFindings

Characteristic:Multipledemyelinatedplaques.Position:Whitematteraroundthelateralventriclesandspinalcord,opticnerve,brainstemandcerebellar.

Acutestage:hyperemia,ondema,demyelination,

infiltriationofinflammatorycellsdistributedinperivenous.Recoverystage:Astrocyteproliferition,formingofastrocyticscab.

4.PathologicFindings

Charact85

急性期:充血、水腫、炎性脫髓鞘、血管周圍Lc浸潤(rùn)。

恢復(fù)期:星狀細(xì)胞增生、膠質(zhì)斑痕形成。

肉眼觀：CNS內(nèi)脫髓鞘斑塊急性期:充血、水腫、炎性脫髓鞘、血管周圍Lc浸865.ClinicalManifestations1)

Prodrome:Thesymptomsevolvedmoreslowly,overseveralweeksormonths.2)Acuteorsubacuteonset(Relapsing-remitting).5.ClinicalManifestations1)873)

Earlysymptomsandsigns:

①Weaknessornumbness;(1/2patientshaveparesthesiaononeormorelimbs)

②

Thevisuallossinoneorbotheyes;③Nystagmus;3)Earlysymptomsandsigns:

884)Commonsymptomsandsigns:①paralysisandparaplegia;②

Thevisuallossinoneorbotheyes;

(1/2patientshavevisualdisorders,relapsing-remitting)

③Nystagmusandpalsyofeyemuscles;(internuclearophthalmoplegia,PPRF

oneandahalfsyndrome)4)Commonsymptomsandsigns:①89“一個(gè)半綜合征”垂直眼震“一個(gè)半綜合征”垂直眼震90脫髓鞘病(7年制)課件91④Sensationdisorder:Romberg’ssign,

(>1/2)Lhermitte’ssign;⑤Ataxia(1/2),Charcot’ssyndrom(laterstage);⑥ImpairmentofPNS;⑦Attacksyndrom;⑧Otherclinicalfeature.④Sensationdisorder:Romberg’926.LaboratoryandassistantTests1)CSFTest

①NumberofMNC<15106/L;②IgG-Index>0.7(70%);oligoclonalbands(OB)(95%);

③MBP,PLP,MAG,MOGAbsandAb-secretingcells;

④CSF-Alb/serum-Alb>1.7(probabilityofMS)6.LaboratoryandassistantTe932)Evokedpotentials:50%-90%abnormal.

①visualevokedpotentials(VEP);②brainstemauditoryevokedpotentials(BAEP);③somatosensoryevokedpotentials(SEP).3)MRI:

①preiventricularplaques;②regularplaquesinbrainstem,cerebellumandspinalcord;③atrophysymptom.2)Evokedpotentials:50%-90%94脫髓鞘病(7年制)課件95脫髓鞘病(7年制)課件96脫髓鞘病(7年制)課件97脫髓鞘病(7年制)課件98脫髓鞘病(7年制)課件99-AbnormalMRIscansarefoundin

96%withadefinitediagnosisofMS

70%withadiagnosisofprobableMS

30-50%withadiagnosisofpossibleMSMRICriteriafordiagnosingMS

Atleast3Lesionsandtwoofthefollowing:1

LesionsabuttingtheLateralVentricles

2Lesionswithdiametersgreaterthan5mm3LesionspresentinthePosteriorFossaSource

(OffenbacherH,FazekasF,SchmidtRetal.

AssessmentOfMRICriteriaForADiagnosisOfMS*Neurology1993;43:905-909)-AbnormalMRIscansarefound100

Diagnosticcriteria1.ClinicaldefiniteMS(CDMS):

①twotimesofattackandtwolesions;②twoattacks,onelesionandonesubclinicalevidence;2.LaboratorysupporteddefiniteMS(LSDMS):

①Twoattacks,onesubclinicalevidenceandCSFOB/IgG;②Oneattack,twolesionsandCSFOB/IgG;③Oneattack,onelesion,onesubclinicalevidenceandCSFOB/IgG;

Diagnosticcriteria1.1013.ClinicalprobableMS(CPMS):

①twoattacks,onelesion;②oneattack,twolesions;③oneattack,onelesionandothersubclinicalevidence;4.LaboratorysupportedprobableMS(LSPMS)

Twoattacks;CSFOB/IgG;TwoattacksinvolvingdifferentpartofCNS,intermissionatlestonemonth;eachattackmustcontinuefor24hs.3.ClinicalprobableMS(CPMS102多發(fā)硬化的診斷標(biāo)準(zhǔn)診斷發(fā)作次數(shù)臨床病灶數(shù)亞臨床證據(jù)CSFOB/IgG臨床確診（CDMS）2221及1實(shí)驗(yàn)室支持確診(LSDMS）211121或1及1+++臨床可能(CPMS)2111211+實(shí)驗(yàn)室支持可能(LSPMS）2兩次發(fā)作均累及CNS不同部位，間隔至少一個(gè)月，每次持續(xù)24小時(shí)。+多發(fā)硬化的診斷標(biāo)準(zhǔn)診斷發(fā)作次數(shù)臨床病灶數(shù)亞臨床證據(jù)CSFO103DifferentialDiagnosis1.急性播散性腦脊髓炎

2.腦動(dòng)脈炎、腦干炎、脊髓血管畸形3.頸椎病脊髓型

4.熱帶痙攣性截癱

5.大腦淋巴瘤DifferentialDiagnosis1.急性播散性104

Treatment目前尚無一種特效療法，治療的主要目的是：1.急性活動(dòng)期抑制其炎癥性脫髓鞘過程，遏止病情的進(jìn)展。

2.盡量預(yù)防能促發(fā)的外因，減少復(fù)發(fā)次數(shù)，延長(zhǎng)緩解間歇期。

3.預(yù)防并發(fā)癥。4.對(duì)癥及支持療法。

Treatment目前尚無一種特效療法，治療的主要目1051.Relapsing-RemittingMS：

①

Anti-inflammatorytreatment:

methylprenisolone(highdosefor3d),prednison,dexamethasone;

②

Suppressionormodulationoftheimmunesystem:IFN-1

and1b

;

Azathioprine；

Immuneglublin(Ig):0.4g/kg.dIVIg3-5d

脫髓鞘病(7年制)課件1062.ProgressiveMS：

①M(fèi)ethotrexate,MTX;

②Cyclosphoamide;③CyclosporineA;④Plasmatransplantation.

2.ProgressiveMS：

①M(fèi)1073.

Symptomatictreatment:

①Spasticity:baclofen,dantrolene,diazepamandtizanidinecanbehelpful.

②Bladderdysfunction:anticholinergicdrugsurinarycathetermayberequired.3.Symptomatictreatment:

108預(yù)后分型1.良性型2.復(fù)發(fā)-緩解3.緩慢進(jìn)展型4.慢性進(jìn)展型預(yù)后分型1.良性型109Examples患者，女，32歲。主訴：行走不穩(wěn)1年，左耳鳴、視物雙影半年。走路不穩(wěn)，踩棉花感左耳鳴復(fù)視快速細(xì)小水平眼震向右凝視時(shí)明顯右側(cè)指鼻試驗(yàn)、輪替試驗(yàn)、跟膝脛試驗(yàn)均欠佳

Romberg征(+)，左Hoffmann征(+)四肢腱反射增高，以雙下肢腱反射增高，右側(cè)踝陣攣陽性頭顱MRI未見異常Examples患者，女，32歲。主訴：行走不穩(wěn)1年，左耳鳴110該患如何定位及定性？該患如何定位及定性？111

視神經(jīng)和脊髓受累較多見，病灶中的軟化、壞死較多見.視神經(jīng)脊髓炎又稱Devic病,為多發(fā)性硬化的一個(gè)亞型.中國(guó),日本等東方人1.Introduction2.EtiologyAndPathogenesis視神經(jīng)脊髓炎

(Neuromyelitisoptica,NMO)

中國(guó),日本1.Introduction2.Etiology1123.Patholgy主要侵犯視神經(jīng)、視交叉、和脊髓胸頸段3.Patholgy主要侵犯視神經(jīng)、視交叉、和脊髓胸頸段113Acutestage:infiltrationofinflammatorycells.AstrocyteproliferitionAcutestag