Review: Creutzfeldt-Jakob disease

Abstract

The clinicopathological spectrum of Creutzfeldt-Jakob disease has recently been extended by the identification of an apparently new variant of this disorder in the UK. The hypothesis that this new disorder is causally related to the transmissible agent responsible for bovine spongiform encephalopathy has prompted re-evaluation of the relationships between human and animal transmissible spongiform encephalopathies, and reinforced the need for adequate experimental models to investigate this field. Recent experimental transmission studies of bovine spongiform encephalopathy into primates have supported the hypothesis, and reinforced the central role of neuropathology in the investigation of this group of diseases. Whilst clearly of distinctive etiology, Creutzfeldt-Jakob disease shares both clinical and neuropathological features with other human dementias; renewed evaluation of potentially similar disease mechanisms in these devastating conditions might provide new information of both scientific and therapeutic value.