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1、中枢神经系统血管炎第1页，此课件共41页哦第2页，此课件共41页哦第3页，此课件共41页哦第4页，此课件共41页哦第5页，此课件共41页哦第6页，此课件共41页哦Classification of CNS vasculitisINFECTIOUS VASCULITIS-Spirochetal(syphilis)-Mycobacterial-Fungal-Rickettsial-Bacterial(purulent)meningitis-Viral-Other organismsNECROTIZING VASCULITIDES-Classic polyarteritis nodosa-Wegen
2、ers granulomatosis-Allergic Angitis and granulomatosis(Churg-Strauss)-Necrotizing systemic vasculitis-overlap syndrome-Lymphomatoid granulomatosisVASCULITIS ASSOCIATED WITH COLLAGEN VASCULAR DISEASES-Systemic lupus erythematosus-Rheumatoid arthritis-Scleroderma-Sjogrens syndromeGIANT CELL ARTERITIDE
3、S-Takayasus arteritis-Temporal(cranial)arteritisVASCULITIS ASSOCIATED WITH OTHER SYSTEMIC DISEASES-Behcets disease-Ulcerative colitis-Sarcoidosis-Relapsing polychondritis-Kohlmeier-Degos diseaseHYPERSENSITIVITY VASCULITIDES-Henoch-Schonlein purpura-Drug-induced vasculitides-Chemical vasculitides-Ess
4、ential mixed cryoglobulinemiaMISCELLANEOUS-Vasculitis associated with neoplasia-Vasculitis associated with radiation-Cogans syndrome-Dermatomyositis-polymyositis-X-linked lymphoproliferative syndrome-Thromboangiitis obliterans-Kawasaki syndromePRIMARY CNS VASCULITIS第7页，此课件共41页哦Historyn n1922 Harbitz
5、s first report.n n1959 Gravioto and Feigins extensive autopsy descriptionsn n1970s Primary CNS angiitis,Granulomatous angiitis of the CNS,isolated CNS angiitis.n n1980s High dose steroid and Cyclophosphamide started.n nPrognosis is very poor without treatment.Mortality is almost 100%without treatmen
6、t第8页，此课件共41页哦Pathology of the isolated CNS vasculitisnThe essential feature is a giant cell,granulomatous inflammation of the small arteries and veins,which exhibits a nearly constant affinity for the vessels of the leptomeninges and the branches that arise from them to penetrate the cortex.nThe siz
7、e is 2-300 micron.第9页，此课件共41页哦Animal Modelsn nIntrvanous injection of Mycoplasma n ngallisepticum in turkeys produced similar n ndamage as human vasculitis.第10页，此课件共41页哦第11页，此课件共41页哦第12页，此课件共41页哦Clinical PresentationAUTOPSYAUTOPSYBIOPSYBIOPSYSYMPTOMS ORSYMPTOMS OR CASES CASES CASESCASES SIGNS SIGNS
8、(N=45)(N=45)(N=26)(N=26)_Altered mentationAltered mentation 393976%76%111142%42%HeadacheHeadache 2929646413135050HemiparesisHemiparesis 20204444111142 42 Stupor or comaStupor or coma 19194242 4 41515DysphasiaDysphasia 1414313111114242SeizuresSeizures 13132929 8 83131“Eye signs”“Eye signs”15153333 3
9、31212ParaparesisParaparesis11112424 4 41515AtaxiaAtaxia 8 81818 9 93535FeverFever 8 81818 3 31212PapilledemaPapilledema 9 92020 1 1 4 4Weight LossWeight Loss 8 81818 0 0 0 0 第13页，此课件共41页哦Diagnostic Testing-1nLabs:CBCtAnti-BM abs,ANCA,ACE,SSA,SSB,FANA,RF,Cryoglobulin,etctESR,C-reactive proteintNormal
10、 ESR for man is age/2,for women is(age+10)/2.tCorrected ESR=ESR (standard Hct-actual Hct)x 1.75.Standard Hct is 45 for man,42 for women.第14页，此课件共41页哦Initial ESR(n=47)nLess than 20 mm/hr 22 47%n21-40 14 30%n41-60 7 15%n61-80 3 6%n 81 1 2%第15页，此课件共41页哦Diagnostic studies for CNS vasculitisTESTSENSITIVI
11、TY ESTIMATED SPECIFICITYCT33-50%Data not available(even lbiopsy-proven cases)no pathognomonic findingsMRI50-100%Data not available(It approaches 100%in histo-no patholognomonic findings logically confirmed cases,and is lowest in those diagnosedonly by angiography)ANGI-30-100%22%ography(It is less th
12、an 40%in Assessed in only one study but histologically confirmed may be higher if vasculitis is cases,and 100%in reports secondary to other causes arenot supported by histology)excluded)BIOPSY75%80%(The negativity can be due The same pattern of inflammation to the patchy nature of the can be due to
13、other causesdisease and small tissuesample 第16页，此课件共41页哦 BillerBiller“VASCULITIS”Look-Alikes on Cerebral AngiographyVASCULITIS”Look-Alikes on Cerebral Angiography_CONDITIONCONDITIONAUTHOR(S)AUTHOR(S)_Neoplastic angioendotheliosisNeoplastic angioendotheliosis Witt et al.Witt et al.Spasm after subarac
14、hnoid hemnorrhageSpasm after subarachnoid hemnorrhageFerris and LevineFerris and LevineAtherosclerosisAtherosclerosisFerris and LevineFerris and LevineOral contraceptive useOral contraceptive use Irey et al.Irey et al.HypertensionHypertension with pheochromocytoma with pheochromocytomaALrmstrong and
15、 Hayes,ALrmstrong and Hayes,Postpartum Postpartum Garner et al.Garner et al.Eclampsia Eclampsia Trommer,Homer,and Trommer,Homer,andMigraineMigraineSchon and HarrisonSchon and HarrisonPostcoital headache (?)Postcoital headache (?)Kapoor,Kendall,Kapoor,Kendall,TraumaTraumaSuwanwela and Suwanwela and S
16、urgical manipulation of intracranial arteriesSurgical manipulation of intracranial arteriesKhodadadKhodadad“Reversible cerebral segmental vasoconstriction”“Reversible cerebral segmental vasoconstriction”Call et al.Call et al.Sumatriptan and isometheptaneSumatriptan and isometheptane第17页，此课件共41页哦Diag
17、nostic Testing-3n nCSF:Very sensitive,but not specificn n90%abnormal第18页，此课件共41页哦Differential Diagnosisn n1.CVA1.CVAn n2.MS2.MSn n3.Infection3.Infectionn n4.Tumor4.Tumorn n5.Specific/systemic vasculitis5.Specific/systemic vasculitisn n6.Toxic6.Toxicn n7.Leukodystrophy7.Leukodystrophyn n8.MERRF,MELAS
18、8.MERRF,MELASn n9.Hypertensive encephalopathy9.Hypertensive encephalopathy第19页，此课件共41页哦第20页，此课件共41页哦7-26-087-26-087-26-087-26-088-3-0882 y/o WF with no PMH admitted because of MS change7-26-087-26-08第21页，此课件共41页哦第22页，此课件共41页哦第23页，此课件共41页哦第24页，此课件共41页哦第25页，此课件共41页哦第26页，此课件共41页哦第27页，此课件共41页哦第28页，此课件共4
19、1页哦第29页，此课件共41页哦第30页，此课件共41页哦第31页，此课件共41页哦第32页，此课件共41页哦第33页，此课件共41页哦第34页，此课件共41页哦第35页，此课件共41页哦第36页，此课件共41页哦MELAS DNA testingMELAS DNA testingMELAS 3243-tRNA leu 3243GMELAS 3271-tRNA leu T3271CMELAS 3252-tRNA leu A3252GMELAS 3256-tRNA leu C3256TMELAS 3291-tRNA leu T3291CMELAS 13,513-ND5 G13513A第37页，此
20、课件共41页哦第38页，此课件共41页哦Treatment for CNS vasculitis CYTOXIC AGENT CORTICOSTEROIDS_Induction Cyclophosphamide 2mg/kd daily Prednisolone 1mg/kg daily therpay by mouth(max 150mg);lower (max 80mg);Reduce weekly to 4 6 mo dose by 25mg if 60 years WBC 10mg/day by 6 months must be 4.0 x 10/1Maintenance Azathi
21、oprine 2mg/kg daily Prednisolone 5-10mg/day therapy6 24 mo MTXEscalation Acute severe disease with creatinine 500 umol/1 or pulmonary therapy hemorrhage;Consider 7 10 plasma exchange treatment over 14 days such that 60 ml/kg of plasma is exchanged for 4.5%or 5%human albumin solution or consider three pulses of methylprednisolone,15 mg/kg daily for 3 days.These patients(if under 60 years)may also require 2.5mg/kg daily of cyclophosphamide.第39页，此课件共41页哦Thank you!第40页，此课件共41页哦第41页，此课件共41页哦