[A rarely recognized cause of cerebral ischemia: cerebral angiitis].

Abstract

Introduction: Inflammation of the walls of the central nervous system blood vessels (angiitis) is a rare cause of a cerebral ischemia. The recognition, diagnosis and treatment of the central nervous system angiitis require a multidisciplinary approach. Objective: Our goal was to write a Hungarian-language summary article and diagnostic protocol to assist clinicians in recognizing and diagnosing central nervous system vasculitis, as well as selecting the appropriate treatment. Discussion: Within central nervous system vasculitis, we distinguish between primary central nervous system vasculitis and secondary forms, where the involvement of the nervous system may be part of another underlying systemic disease. Central nervous system vasculitis can also be categorized based on the diameter of the affected vessels, distinguishing between small, medium, and large vessel types. These forms vary in clinical presentation, and their treatment is also different. Currently, no specific laboratory or imaging biomarkers are available to aid in the diagnosis. Besides physical examination and taking the medical history, it is crucial to utilize routine imaging procedures as well as methods confirming vascular wall involvement (e.g., black blood technique), routine and special laboratory and cerebrospinal fluid tests, and biopsy sampling options. In differential diagnosis, the primary focus is on excluding or confirming systemic vasculitis and autoimmune disorders, as well as infections or other diseases that may also affect the central nervous system. After establishing the diagnosis, the neurologist plays a key role in developing an appropriate treatment strategy, with significant involvement from immunologists/rheumatologists, and close follow-up is recommended due to the progressive nature of the disease. Conclusion: Central nervous system vasculitis is a progressive disease, but in many cases, it is treatable if detected early. Flexible collaboration between medical specialties plays a crucial role in its recognition and treatment. Due to the complexity of diagnosis and long-term care, it may be beneficial to establish specialized centers and working groups skilled in managing the disease. Orv Hetil. 2025; 166(23): 887–906.