Challenging presentation of primary vasculitis of the central nervous system.

Objective: To show a patient with a diagnosis of primary vasculitis of the nervous system and review this entity's literature.

Clinical Case: Male 32 years, with two events; first, with a transient monoparesis of the right upper extremity, improve with steroids in days. Now, with a motor Jacksonian progression from the upper to the lower right extremities and generalized seizures.