中枢神经系统血管炎课件

2023/7/1中枢神经系统血管炎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--KawasakisyndromePRIMARYCNSVASCULITISHistory1922Harbitz’sfirstreport.1959GraviotoandFeigin’sextensiveautopsydescriptions1970sPrimaryCNSangiitis,GranulomatousangiitisoftheCNS,isolatedCNSangiitis.1980sHighdosesteroidandCyclophosphamidestarted.Prognosisisverypoorwithouttreatment.Mortalityisalmost100%withouttreatmentPathologyoftheisolatedCNSvasculitisTheessentialfeatureisagiantcell,granulomatousinflammationofthesmallarteriesandveins,whichexhibitsanearlyconstantaffinityforthevesselsoftheleptomeningesandthebranchesthatarisefromthemtopenetratethecortex.Thesizeis2-300micron.AnimalModelsIntrvanousinjectionofMycoplasmagallisepticuminturkeysproducedsimilardamageashumanvasculitis.

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

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.InitialESR(n=47)Lessthan20mm/hr2247%21-401430%41-60715%61-8036%>8112%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

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 DiagnosticTesting-3CSF:Verysensitive,butnotspecific90%abnormalDifferentialDiagnosis1.CVA2.MS3.Infection4.Tumor5.Specific/systemicvasculitis6.Toxic7.Leukodystrophy8.MERRF,MELAS9.Hypertensiveencephalopathy7-26-087-26-087-26-087-26-088-3-0882y/oWFwithnoPMHadmittedbecauseofMSchange7-26-087-26-08MELASDNAtestingMELAS3243-tRNAleu3243G MELAS3271-tRNAleuT3271CMELAS3252-tRNAleuA3252GMELAS3256-tRNAleuC3256TMELAS3291-tRNAleuT3291CMELAS13,513-ND5G13513ATreatmentforCNSvasculitis CYTOXICAGENT CORTICOSTEROIDS_____________________________________________Induction Cyclophosphamide2mg/kddaily Prednisolone1mg/kgdailytherpay bymouth(max150mg);lower (max80mg);Reduceweeklyto4–6mo doseby25mgif>60yearsWB