[Isolated angiitis of the central nervous system with high immune complex titer, first presenting as intracranial hemorrhage during cesarean section].

A case of isolated angiitis of the central nervous system (IACNS) which first presented as intracranial hemorrhage during cesarean section was reported. The patient was a 27-year-old female who showed severe headache during cesarean section. CT scan disclosed hematoma in the right parieto-occipital area. Carotid and vertebral angiograms demonstrated striking areas of stenosis and irregularity of all intracranial arteries. There was no abnormality in the branches of the external carotid arteries. Systemic angiograms showed no evidence of systemic vasculitis. Biopsy of the temporal artery showed no abnormal findings. Titer of immune complex was elevated. Angiitis showed no improvement with corticosteroid alone, but improved markedly with corticosteroid plus cyclophosphamide. Elevated titer of immune complex became normal. Treatment with a combination of corticosteroid and cyclophosphamide is recommended in IACN.