Transmissible Spongiform Encephalopathies

Hippokratia 1998, 2(3):110-124

N. Flaris, A. Constantinidou


A few decades ago, Creutzfeldt – Jakob disease was an obscure form of dementia, unknown to most physicians. However, today it is a well known disease and the most common form of transmissible spongiform encephalopathies or prion diseases in humans. The recent outbreak of bovine spongiform encephalopathy (madcow disease) in England, the recognition of a new variant of Creutzfeldt – Jakob disease, which is probably related to bovine spongiform encephalopathy, and their presentation by the press and media were mainly responsible for that change. Since 1920, when Creutzfeldt -Jakob disease was first described, until recently the diagnosis of the disease was based on morphological criteria, the most specific of which is the spongiform change of the grey matter. The recognition of the transmissibility of the disease and the identification of prion protein (PrP) as an important element of the responsible infectious agent led to a new classification of the spongiform encephalopathies in humans and animals, and new methods of their diagnosis. Our review will focus on the description of the different forms of the transmissible spongiform encephalopathies, the evolution of our understanding of these diseases during the past decades, and the pathogenetic relationship of the prion protein and these diseases.