中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件

中樞神經(jīng)系統(tǒng)脫髓鞘疾病

DemyelinatingDiseasesoftheCentral

NervousSystem

DepartmentofNeurology2ndhospitalofKunmingMedicalUniversity中樞神經(jīng)系統(tǒng)脫髓鞘疾病

Demyelinating1

Chapter1

Intraduction1.Concept

Agroupofdiseasesofthebrainandspinalcordinwhichdemyelinationisaprominentfeature.Chapter1Intraducti22.PathologicFindings①

Destructionofthemyelinsheathsofnerves;②Infiltrationofinflammatorycellsinaperivasculardistribution;中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件3③Aparticulardistributionoflesion,oftenperivenousandprimarilyinwhitematter,eitherinmultiplesmalldisseminatedfociorinlargerfoci;④Arelativeintegrityoftheaxiscylindersinthelesionsandalackofwallerian,thesecondarydegenerationoffibertracts.③Aparticulardistributionof4多發(fā)性硬化

MultipleSclerosis

1.ConceptMultipleSclerosisisakindofautoimmunediseasescharacterizedbydemyelinationofCNS.Duetoitshighincidence,chronicityandtendencytoattackyoungadults,ithasbecomeoneofthemostimportantdiseasesofCNS.多發(fā)性硬化

MultipleSclerosis

1.Co52.EtiologyAndPathogenesis1)病毒感染及自身免疫反應(yīng)：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、體液免疫(1,2,3,4)2)遺傳因素(inheritedfactor)3)環(huán)境因素(environment)(4)2.EtiologyAndPathogenesis1)63.EpidemiologyIncidenceofMSassociatedwithlatitude.ContactingwithsomekindofenvironmentmayplayanimportantroleintheincidenceofMS.3.EpidemiologyIncidenceofM7Hereditymaybeanimportantfactor.MSassociatedwiththeHLA-DRlocusonthesixthchromosome,HLA-DR2expressstronglyandthen-DR3,

B7andA3.Hereditymaybeanimporta84.

PathologicFindings

Characteristic:MultipledemyelinatedplaquesinwhitematterofCNS.

Position:Whitematteraroundthelateralventriclesandspinalcord,opticnerve,brainstemandcerebellar.

Acutestage:hyperemia,ondema,demyelination,

infiltrationofinflammatorycellsinperivasculardistribution.Recoverystage:Astrocyteproliferition,formingofastrocyticscab.

4.PathologicFindings

Charact95.ClinicalManifestations1)

Prodrome:Thesymptomsevolvedmoreslowly,overseveralweeksormonths.2)AcuteorsubacuteonsetRelapsing-remitting.3)Earlysymptomsandsigns:

①Weaknessornumbness,sometimesboth,inoneormorelimbsistheinitialsymptominabouthalfofthepatients;

5.ClinicalManifestations1)P10②

Thevisuallossinoneorbotheyes;③Nystagmus(fig1)andpalsyofeyemuscles(internuclearophthalmoplegia,

oneandahalfsyndrome)④Impairmentofotherbrainnerves;⑤Ataxia:Charcot’ssyndrom⑥Sensationdisorder:Romberg’ssign,Lhermitte’ssign;⑦

Attacksyndrom⑧Otherclinicalfeature②Thevisuallossinoneorbo11中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件126.LaboratoryandotherassistantTests1.CSFTest1)NumberofMNC<15106/L2)IgG-Index>0.7;oligoclonalbands(OB)positive;3)MBP,PLP,MAG,MOGAbsandAb-secretingcells4)CSF-Alb/serum-Alb>1.7(probabilityofMS)6.Laboratoryandotherassista132.Evokedpotentials:

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

①preiventricularplaques;②regularplaquesinbrainstem,cerebellumandspinalcord;③atrophysymptom.(1,2,3,4,5,6)2.Evokedpotentials:14

Diagnosticcriteria1.ClinicaldefiniteMS(CDMS):

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

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

Diagnosticcriteria1.153.ClinicalprobableMS(CPMS):

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

Twoattacks;CSFOB/IgG;TwoattacksinvolvingdifferentpartofCNS,intermissionatlestonemonth;eachattackmustcontinuefor24hs.3.ClinicalprobableMS(CPMS16DifferentialDiagnosis1.

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17

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?gòu)?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181.Relapsing-RemittingMS：

①ACTHandSteoids：methylprenisolone,prednison,dexamethasone;②IFN-;③Azathioprine；④Immuneglublin（Ig).

中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件192.ProgressiveMS：

①M(fèi)ethotrexate,MTX;

②Cyclosphoamide;③CyclosporineA;④Plasmatransplantation.

3.

對(duì)癥治療及預(yù)防感染避免疲勞：如過度勞累、緊張、疫苗接種、妊娠、分娩等。

2.ProgressiveMS：

①M(fèi)et20視神經(jīng)脊髓炎

(Neuromyelitisoptica,NMO)1.Introduction：AcuteorsubacutedemyelinatingdiseaseinvolvedopticNandspinalcordatthesametime.2.EtiologyAndPathogenesis：與遺傳素質(zhì)和種族差異有關(guān)。西方-腦干；東方-視神經(jīng)和脊髓；25%MS突發(fā)球后視神經(jīng)炎；多方研究證實(shí)白種人對(duì)MS易感；非白種人對(duì)NMO易感。3.Pathology：Demyelination,plaqueofsclerosis,

infiltriationofinflammatorycellsinperivasculardistribution.

視神經(jīng)、視交叉及胸頸段易受累。視神經(jīng)脊髓炎

(Neuromyeli21ClinicalManifestations1、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。ClinicalManifestations1、年輕居多，22一、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23急性播散性腦脊髓炎（ADEM）1、概述：是一種廣泛累及腦和脊髓白質(zhì)的急性炎癥性疾病(感染出疹或疫苗接種)爆發(fā)型：急性出血性白質(zhì)腦炎(AHL)2、病因及發(fā)病機(jī)制：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。3、病理：腦和