Central Nervous System Vasculitis: Immunopathogenesis, Clinical Aspects, and Treatment Options

Inflammation is a naturally occurring phenomenon in stroke and can be either a nonspecific reaction to a vascular insult or a primary component of the pathogenesis. The potential for the inflammation associated with connective tissue disorders to affect the cerebral or spinal circulation is well recognized. Perhaps the most common vasculitic process of the cerebral circulation encountered is temporal, i.e., giant cell arteritis. Determination of a primary component of vascular inflammation in the patient’s presentation can be quite challenging and remains extremely important as it will be vital in determining whether or not immunosuppressive therapy is indicated. Documentation of the vasculitic process in a timely fashion, the need for potent immunosuppressive therapy, as well as the choice and dosing of therapy, along with the duration, are important determinants of outcome in central nervous vasculitis. We hope to provide updated information toward these goals in this chapter.