An internal medicine physician in Elberton, Georgia, Joshua “Josh” Estep, MD, has served with Elbert Memoral Hospital since 2022. Dr. Josh Estep, who earned his MD in 2016, has made significant contributions to research in prion disease, also known as TSE (transmissible spongiform encephalopathy). His experience includes a fellowship at the Prion Research Center at Colorado State University.
Prion disease is a broad category of neurodegenerative disorders that occur in humans and other animals. Quite rare, these disorders share many characteristics, including loss of neurons due to spongiform changes and a reduction in the inflammatory response.
Prion disease takes its name from the pathogenic agents that cause it. Although their exact function remains a scientific mystery, normal prions exist abundantly in the brain without leading to TSE. Abnormally folded prions, however, can reproduce autonomously, spreading biological infection. Generally progressing rapidly, this infection leads to brain damage and death.
Prion disorders that impact humans include Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinker syndrome, and fatal familial insomnia. Among the prion disorders that impact animals, bovine spongiform encephalopathy (“mad cow” disease) affects cattle. and chronic wasting disease affects deer, elk, and moose.
