Abstract

Primary central nervous system (CNS) vasculitis is defined as an inflammation of cerebral vasculature without vasculitis of other organs. It represents a rare and poorly understood form of vascular inflammatory disease restricted to the brain and spinal cord. An average annual incidence rate of 2.4 cases per 1,000,000 persons per year has been reported all over the world. It affects medium and small-sized arteries of the brain parenchyma, spinal cord, and leptomeninges resulting in CNS dysfunction. Here, we report the case of a 19-year-old female brought with a history of headache, fever and vomiting, and altered sensorium, and on further clinical, biochemical and radiological evaluation, a final diagnosis of primary CNS vasculitis was made.

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