Kaustubh Limaye, Edgar A. Samaniego, Harold P. Adams

Primary central nervous system angiitis (PCNSA) is a rare disease. Varying clinical pictures coupled with lack of sensitive and specific diagnostic tests lead to challenges in management of these patients. This unfortunately may lead to both under- or over-diagnosis and unnecessary treatment. It is important to recognize the different conditions that may mimic the clinical and radiologic presentation. We present a contemporary literature review that should update physicians who encounter this patient population. Recent additions to the literature in form of case reports and single-center series show that digital subtraction angiography was the most widely used test to diagnose PCNSA. It is also important to note that brain biopsy is still considered "gold standard" and should be pursued as it not only has information that establishes the diagnosis but also may help in ruling out the diagnosis from mimics. In around 39% of suspected cases, an alternate diagnosis was eventually confirmed highlighting the importance of a comprehensive work-up. For treatment, almost all the literature supports the use of glucocorticoids as the initial treatment and if the patient has a relapse or develops steroid toxicity then the second most commonly used agent was cyclophosphamide. There are increasing reports of benefits with other agents such as methotrexate, azathioprine, mycophenolate, infliximab, and etanercept. The diagnosis and management of PCNSA continues to be a challenge. Formation of prospective patient registries with continued immunologic research for novel targets and immunomodulatory modalities may hold promise for future care of these patients.