中樞神經(jīng)系統(tǒng)血管炎

1、中樞神經(jīng)系統(tǒng)血管炎第一頁(yè)，共三十九頁(yè)。第二頁(yè)，共三十九頁(yè)。第三頁(yè)，共三十九頁(yè)。第四頁(yè)，共三十九頁(yè)。第五頁(yè)，共三十九頁(yè)。Classification of CNS vasculitisINFECTIOUS VASCULITIS- Spirochetal (syphilis)- Mycobacterial- Fungal- Rickettsial- Bacterial (purulent) meningitis- Viral- Other organismsNECROTIZING VASCULITIDES- Classic polyarteritis nodosa- Wegeners granul

2、omatosis- 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-

3、 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 vascul

4、itides- Essential mixed cryoglobulinemiaMISCELLANEOUS- Vasculitis associated with neoplasia- Vasculitis associated with radiation- Cogans syndrome- Dermatomyositis-polymyositis- X-linked lymphoproliferative syndrome- Thromboangiitis obliterans- Kawasaki syndromePRIMARY CNS VASCULITIS第六頁(yè)，共三十九頁(yè)。Histor

5、y1922 Harbitzs first report.1959 Gravioto and Feigins extensive autopsy descriptions1970s Primary CNS angiitis, Granulomatous angiitis of the CNS, isolated CNS angiitis.1980s High dose steroid and Cyclophosphamide started.Prognosis is very poor without treatment. Mortality is almost 100% without tre

6、atment第七頁(yè)，共三十九頁(yè)。Pathology of the isolated CNS vasculitisThe 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.The

7、size is 2-300 micron.第八頁(yè)，共三十九頁(yè)。Animal ModelsIntrvanous injection of Mycoplasma gallisepticum in turkeys produced similar damage as human vasculitis. 第九頁(yè)，共三十九頁(yè)。第十頁(yè)，共三十九頁(yè)。第十一頁(yè)，共三十九頁(yè)。Clinical PresentationAUTOPSYBIOPSYSYMPTOMS OR CASES CASES SIGNS (N = 45) (N = 26)_Altered mentation 3976%1142%Headache 2

8、9641350Hemiparesis 20441142 Stupor or coma 1942 415Dysphasia 14311142Seizures 1329 831“Eye signs”1533 312Paraparesis1124 415Ataxia 818 935Fever 818 312Papilledema 920 1 4Weight Loss 818 0 0 第十二頁(yè)，共三十九頁(yè)。Diagnostic Testing-1Labs: CBCAnti-BM abs, ANCA, ACE, SSA, SSB, FANA, RF, Cryoglobulin, etcESR, C-re

9、active proteinNormal ESR for man is age/2, for women is (age +10)/2.Corrected ESR = ESR (standard Hct-actual Hct) x 1.75. Standard Hct is 45 for man, 42 for women.第十三頁(yè)，共三十九頁(yè)。Initial ESR (n=47)Less than 20 mm/hr 22 47%21-40 14 30%41-60 7 15%61-80 3 6% 81 1 2%第十四頁(yè)，共三十九頁(yè)。Diagnostic studies for CNS vasc

10、ulitisTESTSENSITIVITY 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

11、% 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 patc

12、hy nature of the can be due to other causesdisease and small tissuesample 第十五頁(yè)，共三十九頁(yè)。 Biller“VASCULITIS” Look-Alikes on Cerebral Angiography_CONDITIONAUTHOR(S)_Neoplastic angioendotheliosis Witt et al.Spasm after subarachnoid hemnorrhageFerris and LevineAtherosclerosisFerris and LevineOral contracep

13、tive use Irey et al.Hypertension with pheochromocytomaALrmstrong and Hayes, Postpartum Garner et al. Eclampsia Trommer, Homer, andMigraineSchon and HarrisonPostcoital headache (?)Kapoor, Kendall, TraumaSuwanwela and Surgical manipulation of intracranial arteriesKhodadad“Reversible cerebral segmental

14、 vasoconstriction”Call et al.Sumatriptan and isometheptane第十六頁(yè)，共三十九頁(yè)。Diagnostic Testing-3CSF: Very sensitive, but not specific90% abnormal第十七頁(yè)，共三十九頁(yè)。Differential Diagnosis1. CVA2. MS3. Infection4. Tumor5. Specific / systemic vasculitis6. Toxic7. Leukodystrophy8. MERRF, MELAS9. Hypertensive encephalo

15、pathy第十八頁(yè)，共三十九頁(yè)。第十九頁(yè)，共三十九頁(yè)。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第二十頁(yè)，共三十九頁(yè)。第二十一頁(yè)，共三十九頁(yè)。第二十二頁(yè)，共三十九頁(yè)。第二十三頁(yè)，共三十九頁(yè)。第二十四頁(yè)，共三十九頁(yè)。第二十五頁(yè)，共三十九頁(yè)。第二十六頁(yè)，共三十九頁(yè)。第二十七頁(yè)，共三十九頁(yè)。第二十八頁(yè)，共三十九頁(yè)。第二十九頁(yè)，共三十九頁(yè)。第三十頁(yè)，共三十九頁(yè)。第三十一頁(yè)，共三十九頁(yè)。第三十二頁(yè)，共三十九頁(yè)。第三十三頁(yè)，共三十九頁(yè)。第三十四頁(yè)，共三

16、十九頁(yè)。第三十五頁(yè)，共三十九頁(yè)。MELAS 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第三十六頁(yè)，共三十九頁(yè)。第三十七頁(yè)，共三十九頁(yè)。Treatment for CNS vasculitis CYTOXIC AGENT CORTICOSTEROIDS_Induction Cyclophosphamide 2mg/

17、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 suc