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Classification of CNS vasculitisINFECTIOUS VASCULITIS-Spirochetal(syphilis)-Mycobacterial-Fungal-Rickettsial-Bacterial(purulent)meningitis-Viral-Other organismsNECROTIZING VASCULITIDES-Classic polyarteritis nodosa-Wegeners 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 ARTERITIDES-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-Essential mixed cryoglobulinemiaMISCELLANEOUS-Vasculitis associated with neoplasia-Vasculitis associated with radiation-Cogans syndrome-Dermatomyositis-polymyositis-X-linked lymphoproliferative syndrome-Thromboangiitis obliterans-Kawasaki syndromePRIMARY CNS VASCULITISHistorybb1922 Harbitzs first report.bb1959 Gravioto and Feigins extensive autopsy descriptionsbb1970s Primary CNS angiitis,Granulomatous angiitis of the CNS,isolated CNS angiitis.bb1980s High dose steroid and Cyclophosphamide started.bbPrognosis is very poor without treatment.Mortality is almost 100%without treatmentPathology 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 size is 2-300 micron.Animal ModelsbbIntrvanous injection of Mycoplasma bbgallisepticum in turkeys produced similar bbdamage as human vasculitis.Clinical PresentationAUTOPSYAUTOPSYBIOPSYBIOPSYSYMPTOMS ORSYMPTOMS OR CASES CASES CASESCASES SIGNS SIGNS (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 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 Diagnostic Testing-1nLabs:CBCtAnti-BM abs,ANCA,ACE,SSA,SSB,FANA,RF,Cryoglobulin,etctESR,C-reactive proteintNormal 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.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%Diagnostic studies for CNS vasculitisTESTSENSITIVITY 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 than 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 other causesdisease and small tissuesample 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 subarachnoid 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 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 Surgical 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 isometheptaneDiagnostic Testing-3bbCSF:Very sensitive,but not specificbb90%abnormalDifferential Diagnosisbb1.CVA1.CVAbb2.MS2.MSbb3.Infection3.Infectionbb4.Tumor4.Tumorbb5.Specific/systemic 5.Specific/systemic vasculitisvasculitisbb6.Toxic6.Toxicbb7.7.LeukodystrophyLeukodystrophybb8.MERRF,MELAS8.MERRF,MELASbb9.Hypertensive encephalopathy9.Hypertensive encephalopathy7-26-087-26-087-26-087-26-088-3-0882 y/o WF with no PMH admitted because of MS change7-26-087-26-08MELAS 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 G13513ATreatment 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 Azathioprine 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.Thank you!谢谢！
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