Introduction: Isolated cerebral angiitis of the central nervous system is defined histologically by presence of granulomatous inflammation in the meningeal vessels, and parenchyma throughout veins and arteries of variable size. The most common clinical manifestations are headache and encephalopathy.
Case report: We present the clinical case of a patient with epileptic seizures of focal onset, secondary generalized, diagnosed by cerebral biopsy. Clinical response to immunosuppressive treatment (corticosteroid and cyclophosphamyde) was excellent. We make a bibliographic review and update.
Conclusion: The heterogeneous clinical symptomatology of this disease leads to confusion and delay in diagnosis. Histological confirmation by cerebral and meningeal biopsy is the best parameter for diagnosis of isolated cerebral angiitis of the central nervous system.