中枢神经系统血管炎PPT.ppt

MELAS DNA testing MELAS 3243-tRNA leu 3243G MELAS 3271-tRNA leu T3271C MELAS 3252-tRNA leu A3252G MELAS 3256-tRNA leu C3256T MELAS 3291-tRNA leu T3291C MELAS 13,513-ND5 G13513A 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 /1 Maintenance Azathioprine 2mg/kg daily Prednisolone 5 - 10mg/day therapy 6 – 24 mo MTX Escalation 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! Central Nervous System Vasculitis James Jinxing Wang, MD, PhD Clinical Assistant Professor University of TN Memphis, TN jjxwang@ History 1922 Harbitz’s first report. 1959 Gravioto and Feigin’s extensive autopsy descriptions 1970s 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 treatment Pathology of the isolated CNS vasculitis The 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 size is 2-300 micron. Animal Models Intrvanous injection