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Πρωτοπαθής αγγειίτιδα του κεντρικού νευρικού συστήματος (Master thesis)

Μεχμέτ, Φατμά

Primary central nervous system angiitis (PACNS) is a rare, unexplained condition involving inflammation of small, medium-sized vessels that supply the parenchyma of the brain, spinal cord, and leptomeninges without secondary vascular or systemic evidence. It occurs at any age but most often occurs between 40-50 years. It is an insidious and dangerous condition that requires early diagnosis and treatment to prevent severe disability or even death. Clinical, imaging and laboratory findings are non-specific. Diagnosis requires the combination of clinical/ imaging / laboratory findings, the exclusion of a variety of mimicry conditions while biopsy is the "gold standart" of diagnosis, preferably stereotactic to avoid false negatives. Its treatment, due to the lack of randomized clinical trials so far, prospective studies, is based on the knowledge gained from the trials performed on systemic vasculitis, case reports, retrospective studies and expert opinions. Studies suggest glucocorticoids and cyclophosphamide or mycophenolatemofetil as induction therapy while mycophenolatemofetil, azathioprine and methotrexate are good choices for maintenance therapy. Good results from the use of biological agents (rituximab and TNF-α inhibitors) have been reported in more aggressive cases, in cases of intolerance or non-response to previous treatment. The subject of this postgraduate work is the review of the literature on the diagnostic investigation and treatment of primary angiitis of the central nervous system.