Diffuse inflammation of small- and medium-sized blood vessels confined exclusively to the brain, meninges, or spinal cord is called primary angiitis of the central nervous system (PACNS). There have been significant advances in the understanding of this disorder due to the identification of pathological and clinical subsets, along with advances in the imaging modality of vessel wall abnormalities. A considerable gap still exists, however, particularly with respect to its pathophysiological mechanisms. Although various laboratory and neuroimaging findings may support the diagnosis of primary CNS angiitis, they are not highly specific, and neurologists often face a substantial challenge when diagnosing this disorder because they need to rule out a vast array of nonvasculitic conditions and secondary causes of central nervous system vasculitis. Therefore, a correct and timely diagnosis of primary CNS angiitis requires a high degree of suspicion coupled with knowledge of other diseases that can masquerade as primary CNS vasculitis.
In this article, Dr. Jorge Moncayo-Gaete of the International University of Ecuador and Dr. Julien Bogousslavsky of the Genolier Swiss Medical Network focus on the clinical and pathological findings, diagnostic work-up, differential diagnosis, and current therapeutic options of this still enigmatic and complex entity.
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