A Look at Prion Diseases

 An accomplished internal medicine physician, Joshua “Josh” Estep, MD, served as a hospitalist at Samaritan Pacific Communities Hospital in Newport, Oregon, where he provided high-quality care to patients in a rural setting. Leveraging his experience, he now serves as an internal medicine hospitalist at Elbert Memorial Hospital in Elberton, Georgia. Dr. Josh Estep also has extensive experience in rural medicine and prion disease research.

A group of rare diseases, prion diseases occur when certain proteins called prions accumulate in the brain. Prions alter the structures of other proteins in brain cells and often lead to adverse symptoms such as confusion, muscle stiffness, hallucinations, difficulty walking, and difficulty speaking, depending on what parts of the brain are compromised by the disease. Examples of prion diseases are Creutzfeldt-Jakob disease (CJD), kuru, fatal insomnia, Gerstmann-Straussler-Scheinker disease (GSS), and variably protease-sensitive prionopathy (VPSPr).

Roughly 300 prion disease cases are reported annually in the US, according to hopkinsmedicine.org. A prion disease can be inherited from parents or acquired through environmental factors like exposure to prion-contaminated medical equipment. Some types of prion diseases affect animals, such as cattle. If a human eats prion-contaminated meat, they can also acquire the disease. Prion diseases are generally fatal, and often result in death within a year. While prion diseases are incurable, medical management can delay disease progression and alleviate symptoms.