中枢神经系统血管炎

中枢神经系统血管炎目前一页\总数三十九页\编于十五点目前二页\总数三十九页\编于十五点目前三页\总数三十九页\编于十五点目前四页\总数三十九页\编于十五点目前五页\总数三十九页\编于十五点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目前六页\总数三十九页\编于十五点History1922Harbitz’sfirstreport.1959GraviotoandFeigin’sextensiveautopsydescriptions1970sPrimaryCNSangiitis,GranulomatousangiitisoftheCNS,isolatedCNSangiitis.1980sHighdosesteroidandCyclophosphamidestarted.Prognosisisverypoorwithouttreatment.Mortalityisalmost100%withouttreatment目前七页\总数三十九页\编于十五点PathologyoftheisolatedCNSvasculitisTheessentialfeatureisagiantcell,granulomatousinflammationofthesmallarteriesandveins,whichexhibitsanearlyconstantaffinityforthevesselsoftheleptomeningesandthebranchesthatarisefromthemtopenetratethecortex.Thesizeis2-300micron.目前八页\总数三十九页\编于十五点AnimalModelsIntrvanousinjectionofMycoplasmagallisepticuminturkeysproducedsimilardamageashumanvasculitis.

目前九页\总数三十九页\编于十五点目前十页\总数三十九页\编于十五点目前十一页\总数三十九页\编于十五点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

目前十二页\总数三十九页\编于十五点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.目前十三页\总数三十九页\编于十五点InitialESR(n=47)Lessthan20mm/hr2247%21-401430%41-60715%61-8036%>8112%目前十四页\总数三十九页\编于十五点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

目前十五页\总数三十九页\编于十五点

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 目前十六页\总数三十九页\编于十五点DiagnosticTesting-3CSF:Verysensitive,butnotspecific90%abnormal目前十七页\总数三十九页\编于十五点DifferentialDiagnosis1.CVA2.MS3.Infection4.Tumor5.Specific/systemicvasculitis6.Toxic7.Leukodystrophy8.MERRF,MELAS9.Hypertensiveencephalopathy目前十八页\总数三十九页\编于十五点目前十九页\总数三十九页\编于十五点7-26-087-26-087-26-087-26-088-3-0882y/oWFwithnoPMHadmittedbecauseofMSchange7-26-087-26-08目前二十页\总数三十九页\编于十五点目前二十一页\总数三十九页\编于十五点目前二十二页\总数三十九页\编于十五点目前二十三页\总数三十九页\编于十五点目前二十四页\总数三十九页\编于十五点目前二十五页\总数三十九页\编于十五点目前二十六页\总数三十九页\编于十五点目前二十七页\总数三十九页\编于十五点目前二十八页\总数三十九页\编于十五点目前二十九页\总数三十九页\编于十五点目前三十页\总数三十九页\编于十五点目前三十一页\总数三十九页\编于十五点目前三十二页\总数三十九页\编于十五点目前三十三页\总数三十九页\编于十五点目前三十四页\总数三十九页\编于十五点目前三十五页\总数三十九页\编于十五点MELASDNAtestingMELAS3243-tRNAleu3243G MELAS3271-tRNAleuT3271CMELAS3252-tRNAleuA3252GMELAS3256-tRNAleuC3256TMELAS3291-tRNAleuT3291CMELAS13,513-ND5G13513A目前三十六页\总数三十九页\编于十五点目前三十七页\总数三十九页\编于十五点TreatmentforCNSvasculitis

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