A Case Report of Primary Angiitis of the Central Nervous System

Abstract

BACKGROUND AND OBJECTIVE: Primary angiitis of the central nervous system (PACNS) is an inflammatory vasculitis with very low frequency and prevalence. It is not clear why the inflammatory process of this disease is limited to cerebrovascular disease without systemic manifestations. A case of primary angiitis of the central nervous system with cerebrovascular manifestations is reported here. CASE REPORT: The patient is a 50-year-old woman with headache, nausea, vomiting, and visual symptoms without clear motor impairment. Normocytic normochromic anemias tests and function tests of kidney, liver and thyroid and serology of infectious diseases were reported to be normal. The patient underwent brain imaging, and due to multiple lesions in both cerebral hemispheres and cerebellum, she underwent biopsy with a probable diagnosis of cerebral vasculitis. After histopathologic confirmation, the patient was discharged with glucocorticoid and cyclophosphamide therapy, and the symptoms subsided after one year follow-up. CONCLUSION: Based on the results of this case report, some general and common symptoms of the primary angiitis of the central nervous system should also be considered.