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1、中枢神经系统血管炎第1页，本讲稿共41页第2页，本讲稿共41页第3页，本讲稿共41页第4页，本讲稿共41页第5页，本讲稿共41页第6页，本讲稿共41页Classification of CNS vasculitisINFECTIOUS VASCULITIS-Spirochetal(syphilis)-Mycobacterial-Fungal-Rickettsial-Bacterial(purulent)meningitis-Viral-Other organismsNECROTIZING VASCULITIDES-Classic polyarteritis nodosa-Wegeners gr
2、anulomatosis-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-Taka
3、yasus 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
4、 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，本讲稿共41页Historyn n1922 Harbitzs first
5、 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 treatment第8页，本讲
6、稿共41页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 size is 2-3
7、00 micron.第9页，本讲稿共41页Animal Modelsn nIntrvanous injection of Mycoplasma n ngallisepticum in turkeys produced similar n ndamage as human vasculitis.第10页，本讲稿共41页第11页，本讲稿共41页第12页，本讲稿共41页Clinical PresentationAUTOPSYAUTOPSYBIOPSYBIOPSYSYMPTOMS ORSYMPTOMS OR CASES CASES CASESCASES SIGNS SIGNS (N=45)(N=45)
8、(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 31212Parapar
9、esisParaparesis11112424 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，本讲稿共41页Diagnostic Testing-1nLabs:CBCtAnti-BM abs,ANCA,ACE,SSA,SSB,FANA,RF,Cryoglobulin,etctESR,C-reactive proteintNormal ESR for man
10、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，本讲稿共41页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，本讲稿共41页Diagnostic studies for CNS vasculitisTEST SENSITIVITY ESTIMATED S
11、PECIFICITYCT33-50%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 Asses
12、sed 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 causesdi
13、sease and small tissuesample 第16页，本讲稿共41页 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 hemnorrha
14、geSpasm 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,ALrmstro
15、ng 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 manipul
16、ation 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，本讲稿共41页Diagnostic Testing-3
17、n nCSF:Very sensitive,but not specificn n90%abnormal第18页，本讲稿共41页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,MELAS8.MERRF,MELASn n9
18、.Hypertensive encephalopathy9.Hypertensive encephalopathy第19页，本讲稿共41页第20页，本讲稿共41页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，本讲稿共41页第22页，本讲稿共41页第23页，本讲稿共41页第24页，本讲稿共41页第25页，本讲稿共41页第26页，本讲稿共41页第27页，本讲稿共41页第28页，本讲稿共41页第29页，本讲稿共41页第30页，本讲稿共41页
19、第31页，本讲稿共41页第32页，本讲稿共41页第33页，本讲稿共41页第34页，本讲稿共41页第35页，本讲稿共41页第36页，本讲稿共41页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，本讲稿共41页第38页，本讲稿共41页Treatment for CNS
20、 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 5-10
21、mg/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，本讲稿共41页Thank you!第40页，本讲稿共41页第41页，本讲稿共41页