Takayasu arteritis (TAK) is a systemic vasculitis involving large arteries. Reports of direct central nervous system (CNS) involvement in TAK are extremely rare in the literature. In addition, treatment for direct involvement has not been reported. Herein, we describe a case of encephalitis in a TAK patient who presented with fever and headache at the first attack, then cognitive impairment at the second attack. The patient improved with rituximab and especially infliximab. These findings indicate the usefulness of rituximab and infliximab to treat the direct CNS manifestations in TAK.
