Abstract
Angiitis of the central nervous system (ACNS) is a rather new disease entity that is defined as vascular inflammation limited to the central nervous system and was formally nominated in 1959. Etiologically, it can be divided into primary and secondary ACNS. However, the potential pathogenesis of ACNS remains unclear. The clinical presentation is variable, and there is no consensus concerning its diagnosis and management. Although the incidence is relatively low, ACNS is still a life-threatening condition. It is essential to get a comprehensive and updated understanding of the disease. In this paper, we reviewed the history, definition, classification, pathogenesis, and clinical manifestations of ACNS. In addition, we focused on the latest investigations and viewpoints regarding the diagnosis and treatment of ACNS.