Primary Angiitis of Central Nervous System: A Case Report and Literature Review

Abstract

Primary angiitis of central nervous system (PACNS) is a rare form of vasculitis. We described a 34-year-old women with initial presentation of acute stroke symptoms. Her neurological deficits were in progression under the treatment of ischemic stroke. The possible etiologies in young stroke including congenital or acquired heart disease, hematological disorder, coagulopathies, congenital metabolic anormalies and CNS infection, were all excluded after work-up. Angiography showed irregular narrowing of the lumen of bilateral PCA and SCA and arteritis was highly suspected by the image study. She was treated with the combination pulse therapy of corticosteroid and cyclophosphamide successfully except some neurological sequels left. Here, we review and discuss the diagnosis and treatment of PACNS.