中枢神经系统血管炎

中枢神经系统血管炎现在是1页\一共有39页\编辑于星期二现在是2页\一共有39页\编辑于星期二现在是3页\一共有39页\编辑于星期二现在是4页\一共有39页\编辑于星期二现在是5页\一共有39页\编辑于星期二ClassificationofCNSvasculitisINFECTIOUSVASCULITIS--Spirochetal(syphilis)--Mycobacterial--Fungal--Rickettsial--Bacterial(purulent)meningitis--Viral--OtherorganismsNECROTIZINGVASCULITIDES--Classicpolyarteritisnodosa--Wegener’sgranulomatosis--AllergicAngitisandgranulomatosis(Churg-Strauss)--Necrotizingsystemicvasculitis-overlapsyndrome--LymphomatoidgranulomatosisVASCULITISASSOCIATEDWITHCOLLAGENVASCULARDISEASES--Systemiclupuserythematosus--Rheumatoidarthritis--Scleroderma--Sjogren’ssyndromeGIANTCELLARTERITIDES--Takayasu’sarteritis--Temporal(cranial)arteritisVASCULITISASSOCIATEDWITHOTHERSYSTEMICDISEASES--Behcet’sdisease--Ulcerativecolitis--Sarcoidosis--Relapsingpolychondritis--Kohlmeier-DegosdiseaseHYPERSENSITIVITYVASCULITIDES--Henoch-Schonleinpurpura--Drug-inducedvasculitides--Chemicalvasculitides--EssentialmixedcryoglobulinemiaMISCELLANEOUS--Vasculitisassociatedwithneoplasia--Vasculitisassociatedwithradiation--Cogan’ssyndrome--Dermatomyositis-polymyositis--X-linkedlymphoproliferativesyndrome--Thromboangiitisobliterans--KawasakisyndromePRIMARYCNSVASCULITIS现在是6页\一共有39页\编辑于星期二History1922Harbitz’sfirstreport.1959GraviotoandFeigin’sextensiveautopsydescriptions1970sPrimaryCNSangiitis,GranulomatousangiitisoftheCNS,isolatedCNSangiitis.1980sHighdosesteroidandCyclophosphamidestarted.Prognosisisverypoorwithouttreatment.Mortalityisalmost100%withouttreatment现在是7页\一共有39页\编辑于星期二PathologyoftheisolatedCNSvasculitisTheessentialfeatureisagiantcell,granulomatousinflammationofthesmallarteriesandveins,whichexhibitsanearlyconstantaffinityforthevesselsoftheleptomeningesandthebranchesthatarisefromthemtopenetratethecortex.Thesizeis2-300micron.现在是8页\一共有39页\编辑于星期二AnimalModelsIntrvanousinjectionofMycoplasmagallisepticuminturkeysproducedsimilardamageashumanvasculitis.

现在是9页\一共有39页\编辑于星期二现在是10页\一共有39页\编辑于星期二现在是11页\一共有39页\编辑于星期二ClinicalPresentation

AUTOPSY BIOPSYSYMPTOMSOR CASES CASESSIGNS (N=45) (N=26)_____________________________________________________________________

Alteredmentation 39 76% 11 42%Headache 29 64 13 50Hemiparesis 20 44 11 42Stupororcoma 19 42 4 15Dysphasia 14 31 11 42Seizures 13 29 8 31“Eyesigns” 15 33 3 12Paraparesis 11 24 4 15Ataxia 8 18 9 35Fever 8 18` 3 12Papilledema 9 20 1 4WeightLoss 8 18 0 0

现在是12页\一共有39页\编辑于星期二DiagnosticTesting-1Labs:CBCAnti-BMabs,ANCA,ACE,SSA,SSB,FANA,RF,Cryoglobulin,etcESR,C-reactiveproteinNormalESRformanisage/2,forwomenis(age+10)/2.CorrectedESR=ESR–(standardHct-actualHct)x1.75.StandardHctis45forman,42forwomen.现在是13页\一共有39页\编辑于星期二InitialESR(n=47)Lessthan20mm/hr2247%21-401430%41-60715%61-8036%>8112%现在是14页\一共有39页\编辑于星期二DiagnosticstudiesforCNSvasculitisTEST SENSITIVITY ESTIMATEDSPECIFICITYCT 33-50% Datanotavailable (evenlbiopsy-provencases) nopathognomonicfindingsMRI 50-100% Datanotavailable (Itapproaches100%inhisto-nopatholognomonicfindings logicallyconfirmedcases, andislowestinthosediagnosed onlybyangiography)ANGI- 30-100% 22%ography (Itislessthan40%inAssessedinonlyonestudybut histologicallyconfirmedmaybehigherifvasculitisis cases,and100%inreportssecondarytoothercausesare notsupportedbyhistology)excluded)BIOPSY 75% 80% (Thenegativitycanbedue Thesamepatternofinflammation tothepatchynatureofthe canbeduetoothercauses diseaseandsmalltissue sample

现在是15页\一共有39页\编辑于星期二

Biller“VASCULITIS”Look-AlikesonCerebralAngiography

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CONDITION AUTHOR(S)

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Neoplasticangioendotheliosis Wittetal.

Spasmaftersubarachnoidhemnorrhage FerrisandLevine

Atherosclerosis FerrisandLevine

Oralcontraceptiveuse Ireyetal.

Hypertension

withpheochromocytoma ALrmstrongandHayes,

Postpartum Garneretal.

Eclampsia Trommer,Homer,and

Migraine SchonandHarrison

Postcoitalheadache(?) Kapoor,Kendall,

Trauma Suwanwelaand

Surgicalmanipulationofintracranialarteries Khodadad

“Reversiblecerebralsegmentalvasoconstriction” Calletal.

Sumatriptanandisometheptane 现在是16页\一共有39页\编辑于星期二DiagnosticTesting-3CSF:Verysensitive,butnotspecific90%abnormal现在是17页\一共有39页\编辑于星期二DifferentialDiagnosis1.CVA2.MS3.Infection4.Tumor5.Specific/systemicvasculitis6.Toxic7.Leukodystrophy8.MERRF,MELAS9.Hypertensiveencephalopathy现在是18页\一共有39页\编辑于星期二现在是19页\一共有39页\编辑于星期二7-26-087-26-087-26-087-26-088-3-0882y/oWFwithnoPMHadmittedbecauseofMSchange7-26-087-26-08现在是20页\一共有39页\编辑于星期二现在是21页\一共有39页\编辑于星期二现在是22页\一共有39页\编辑于星期二现在是23页\一共有39页\编辑于星期二现在是24页\一共有39页\编辑于星期二现在是25页\一共有39页\编辑于星期二现在是26页\一共有39页\编辑于星期二现在是27页\一共有39页\编辑于星期二现在是28页\一共有39页\编辑于星期二现在是29页\一共有39页\编辑于星期二现在是30页\一共有39页\编辑于星期二现在是31页\一共有39页\编辑于星期二现在是32页\一共有39页\编辑于星期二现在是33页\一共有39页\编辑于星期二现在是34页\一共有39页\编辑于星期二现在是35页\一共有39页\编辑于星期二MELASDNAtestingMELAS3243-tRNAleu3243G MELAS3271-tRNAleuT3271CMELAS3252-tRNAleuA3252GMELAS3256-tRNAleuC3256TMELAS3291-tRNAleuT3291CMELAS13,513-ND5G13513A现在是36页\一共有39页\编辑于星期二现在是37页\一共有39页\编辑于星期二TreatmentforCNSvasculitis

CYTOXICAGENT CORTICOSTEROIDS_____________________________________________Induction Cyclophosphamide2mg/kddaily Prednisolone1mg/kgdailytherpay bymouth(max150mg);lower (max80mg);Reduceweeklyto4–6mo