Primary Angiitis of the Central Nervous System: An Uncommon Cause of Stroke in the Young

Abstract

Primary angiitis of the central nervous system (PACNS) usually presents with symptoms of headache, cognitive impairment, or stroke with a mean age of onset at 50 years. Inflammation of the cerebral vessels can cause narrowing, occlusion, or thrombosis resulting in tissue ischemia and necrosis of the involved vessel territory. Findings can be seen on Magnetic Resonance Imaging (MRI) scans of the brain and serological markers of inflammation are typically within normal limits. The nonspecificity of PACNS presents a challenge for accurate diagnosis and must be differentiated from secondary vasculitis and Reversible Cerebral Vasoconstriction Syndrome (RCVS). Cerebral angiography, even though having low sensitivity and specificity, could sometimes be the only diagnostic tool available.