Primary angiitis of the central nervous system – A challenging diagnosis

• Published in: Journal of neuroimmunology 2022-05, Vol.366, p.577844-577844, Article 577844
• Main Authors: Paul, Shabeer Ahmad, Roy, Devlina, Mondal, Gouranga Prasad, Bhattacharyya, Ramesh, Ghosh, Kartik Chandra, Das, Sarbajit, Krishna, Hema, Patra, Chandrakanta, Kiran, Jyoti, Benito-León, Julián
• Format: Article
• Language: English
• Subjects: Central nervous system; Central Nervous System - pathology; Cyclophosphamide; Headache; Humans; Primary angiitis of the central nervous system; Review; Vasculitis; Vasculitis, Central Nervous System - diagnostic imaging
• ISSN: 0165-5728; 1872-8421
• DOI: 10.1016/j.jneuroim.2022.577844

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. •PACNS is mimicked by a vast number of inflammatory and non-inflammatory vascular disorders.•Brain biopsy is of essential importance in confirming the diagnosis.•Newer MRI imaging protocols like vessel wall imaging may prove to have a supplemental role in supporting the diagnosis of vasculitis.•Induction regimens with rituximab can prove to be an alternative to cyclophosphamide.