Primary angiitis of central nervous system - A challenging diagnosis

Primary angiitis of the central nervous system is a rare disease characterized by vasculitis of the central nervous system without any systemic involvement. This review aims to provide an insight into the existing stagnancies in the diagnostic approach and management of this disease. The clinical presentation is typically nonspecific, ranging from headaches, altered sensorium, and seizures to recurrent ischemic strokes. The definitive diagnosis can only be ascertained by histopathological studies of tissue obtained from a brain biopsy. While angiography can provide clues to diagnosis, it has often been normal, even in biopsy-proven cases. Primary angiitis of the central nervous system continues to be a diagnostic challenge as little progress has been made over the years in the diagnosis and management strategies. Considering the vast list of mimickers of primary angiitis of the central nervous system and the existence of a significant proportion of imaging-negative and biopsy-negative cases, it becomes imperative to devise universally accepted diagnostic criteria for this disease. Steroids in combination with cyclophosphamide are the agents used to achieve remission. Rituximab can be an alternative. The treatment-related toxicity of cyclophosphamide warrants larger trials for alternative drugs to be studied.