Rapidly progressive dementia disclosing primary angiitis of the central nervous system

Angiitis of the central nervous system involves a wide spectrum of clinical symptoms. Complementary exams (CSF, neuroradiological exams) are not very specific and sensitive. Diagnostic procedures often require cortical and le ptomeningeal biopsy. Treatment, based on corticosteroids and cyclophosphami d, have greatly improved prognosis. We report a rapidly progress dementia s yndrome that revealed angiitis of the central nervous system. Corticosteroi d treatment did not prevent a fatal outcome. Autopsy findings confirmed the diagnosis of central nervous leucocytoclastic angiitis.