title: [Diagnosis and differential cerebral vasculitis diagnosis].
abstract: Over a 5-year period, we investigated 77 consecutive patients (36 males, 41 females, mean age 40.9 years) referred to our hospital with the diagnosis of CNS vasculitis. Extensive workup including MRI, echocardiography, laboratory tests, angiography ( n=53), and biopsies at appropriate sites ( n=26) was performed based on individual history and symptoms. Prominent symptoms were stroke ( n=61), encephalopathy ( n=14), and headaches ( n=2). Vasculitis was finally diagnosed in 13 patients (17%) including isolated angiitis of the CNS ( n=3), giant cell arteritis ( n=4), and septic arteritis ( n=3). Thirty-two patients (42%) presented noninflammatory vasculopathies including moyamoya ( n=6), Sneddon's syndrome ( n=5), dissection ( n=4), CADASIL ( n=2), and collagen vascular disease ( n=9). Coagulopathy was found in 14 cases (18%) including antiphospholipid syndrome ( n=8) and APC resistance ( n=4). Other causes were cardiogenic embolism ( n=8), multiple sclerosis ( n=5), and migraine stroke ( n=3). Only a minority of patients referred for evaluation of suspected CNS vasculitis actually present with inflammatory vascular disease. Main differential diagnosis includes noninflammatory vasculopathies, coagulopathies, and cardiac disease. Since septic processes may be responsible for the symptoms, "blind" treatment with immunosuppressive agents should be strictly avoided.
authors.affiliation: Klinik für Neurologie mit Klinischer Neurophysiologie, Alfried-Krupp-Krankenhaus, Essen. peter.berlit@krupp-krankenhaus.de
authors.forename: P
authors.initials: P
authors.lastname: Berlit
mesh_terms: D000328:Adult; D001925:Brain Damage, Chronic / Q000175:diagnosis / Q000209:etiology; D000075202:Contraindications; D003937:Diagnosis, Differential; D003952:Diagnostic Imaging; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000009:adverse effects; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination; D012189:Retrospective Studies; D012307:Risk Factors; D020293:Vasculitis, Central Nervous System / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology
chemical_list: D007166:Immunosuppressive Agents