This chapter provides an updated review on the ongoing surveillance and diagnosis of prion disease, also termed transmissible spongiform encephalopathy. While prion disease occurs in both animals and humans, this review focuses on human prion disease surveillance. It includes an overview of human prion disease, current diagnostic criteria and emerging technologies, surveillance centers around the world, especially the United States, epidemiology, and clinical and pathological features of cases examined in recent years. © 2006 Elsevier Inc. All rights reserved.
