中枢神经系统血管炎

Central Nervous System VasculitisJames Jinxing Wang, MD, PhDClinical Assistant ProfessorUniversity of TNMemphis, TNClassification 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 VASCULITISHistoryb1922 Harbitzs first report.b1959 Gravioto and Feigins extensive autopsy descriptionsb1970s Primary CNS angiitis, Granulomatous angiitis of the CNS, isolated CNS angiitis.b1980s High dose steroid and Cyclophosphamide started.bPrognosis 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 ModelsbIntrvanous injection of Mycoplasma bgallisepticum in turkeys produced similar bdamage as human vasculitis. Clinical PresentationAUTOPSY BIOPSYSYMPTOMS OR CASES CASESSIGNS (N = 45) (N = 26)_Altered mentation 39 76% 11 42%Headache 29 64 13 50Hemiparesis 20 44 11 42 Stupor or coma 19 42 4 15Dysphasia 14 31 11 42Seizures 13 29 8 31“Eye signs” 15 33 3 12Paraparesis 11 24 4 15Ataxia 8 18 9 35Fever 8 18 3 12Papilledema 9 20 1 4Weight Loss 8 18 0 0Diagnostic Testing-1n Labs: CBCt Anti-BM abs, ANCA, ACE, SSA, SSB, FANA, RF, Cryoglobulin, etct ESR, C-reactive proteint Normal ESR for man is age/2, for women is (age +10)/2.t Corrected 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 vasculitisTEST SENSITIVITY ESTIMATED SPECIFICITYCT 33-50% Data not available(even lbiopsy-proven cases) no pathognomonic findingsMRI 50-100% Data not available(It approaches 100% in histo- no patholognomonic findingslogically 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 buthistologically confirmed may be higher if vasculitis iscases, and 100% in reports secondary to other causes arenot supported by histology) excluded)BIOPSY 75% 80%(The negativity can be due The same pattern of inflammationto the patchy nature of the can be due to other causesdisease and small tissuesampleBiller“VASCULITIS” Look-Alikes on Cerebral Angiography_CONDITION AUTHOR(S)_Neoplastic angioendotheliosis Witt et al.Spasm after subarachnoid hemnorrhage Ferris and LevineAtherosclerosis Ferris and LevineOral contraceptive use Irey et al.Hypertensionwith pheochromocytoma ALrmstrong and Hayes,Postpartum Garner et al.Eclampsia Trommer, Homer, andMigraine Schon and HarrisonPostcoital headache (?) Kapoor, Kendall, Trauma Suwanwela and Surgical manipulation of intracranial arteries Khodadad“Reversible cerebral segmental vasoconstriction” Call et al.Sumatriptan and isometheptane Diagnostic Testing-3bCSF: Very sensitive, but not specificb90% abnormalDifferential Diagnosisb 1. CVAb 2. MSb 3. In