Table of Contents
ISRN Infectious Diseases
Volume 2013 (2013), Article ID 387925, 11 pages
http://dx.doi.org/10.5402/2013/387925
Review Article

Transmissible Spongiform Encephalopathies Affecting Humans

Department of Pharmacology & Toxicology, College of Veterinary Science & Animal Husbandry, Sardarkrushinagar Dantiwada Agricultural University, Sardarkrushinagar 385506, Gujarat, India

Received 2 April 2012; Accepted 5 May 2012

Academic Editors: A. Carvalho, K. Peoc'H, and T. A. Rupprecht

Copyright © 2013 Dudhatra G. B. et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Transmissible spongiform encephalopathies (TSEs) or prion diseases are group of rare and rapidly progressive fatal neurologic diseases. The agents responsible for human prion diseases are abnormal proteins or prion that can trigger chain reactions causing normal proteins in the brain to change to the abnormal protein. These abnormal proteins are resistant to enzymatic breakdown, and they accumulate in the brain, leading to damage. TSEs have long incubation periods followed by chronic neurological disease and fatal outcomes, have similar pathology limited to the CNS including convulsions, dementia, ataxia, and behavioral or personality changes, and are experimentally transmissible to some other species.