Question

what are prion disease? what causes them, what is their cellular pathology, and what are their signs and symptoms?

Answer 1

Prion diseases are a type of neurodegenerative diseases caused by prions, which are “proteinaceous infectious particles.”

These diseases are caused due to misfolded forms of the prion protein (PrP). Such diseases affect a lot of mammals in addition to humans. These include scrapie in sheep, chronic wasting disease in deer, and mad cow disease in cows. In human beings prion diseases occur in various types like fatal familial insomnia , kuru, and Gertsmann-Straussler-Scheinker syndrome.

In such diseases, PrP folds up in wrong way and becomes a prion. It then causes other PrP molecules to do the same i,e fold wrongly. Prions can spread “silently” across a person’s brain for years without any symptoms. In the end they start killing neurons, and once symptoms start to show, the person has a very rapid cognitive decline. These diseases are mostly fatal within a few months, though some can last a few years.

Symptoms of prion diseases:

1). Rapidly developing dementia

2). Confusion and fatigue

3). Hallucinations

4). Walking difficulty, muscle stiffness and speaking difficulty