Introduction:

primary vasculitis of the central nervous system (PVCNS) is rare, with heterogeneous clinical presentation.

Case report:

35-years-old female with seven days with headache, without improvement with analgesics, adding vomiting and stupor, right hemiparesis, and aphasia; CT showed hemorrhages in the right caudate nucleus and left frontal lobe, laboratory tests including antibodies and acute phase reactants normal; pan-angiography showed: a pattern of vasculitis of trunk branches in cerebral arteries and occlusion of the inferior longitudinal sinus; with a diagnosis of possible PVCNS. Drainage of frontal hemorrhage and biopsy was performed, which confirmed the diagnosis; beginning treatment with methylprednisolone bolus and cyclophosphamide, obtaining clinical improvement.

Conclusions:

PVCNS presents heterogeneous clinical manifestations of acquired neurological deficits; it is a challenge. Its diagnosis implies the existence of the following: a) the acquired neurological deficit, unexplained by another cause, b) angiographic or histopathological evidence of PVCNS, c) absence of systemic vasculitis or other condition that explains it.