Abstract
A 52-year-old woman known to have anti-synthetase syndrome (ASS) with positive anti-alanyl-tRNA synthetase antibody (anti-PL 12) for 4 years presented with headache and progressive deterioration of cognitive functions manifested predominantly by episodes of confusion and dyslexia. Clinical, laboratory and radiological evaluation as well as response to treatment was indicative of vasculitis of the central nervous system (CNS). CNS vasculitis is one of the rare manifestations of inflammatory myositis and no case has been reported to suggest CNS vasculitis in ASS.