Prion Diseases Overview

<p>MRI brain scan (C)</p> Signup and view all the answers

<p>Inherited Prion Disease (IPD) (A)</p> Signup and view all the answers

<p>It is produced by the PRNP gene and protects brain cells. (D)</p> Signup and view all the answers

<p>Akinetic mutism. (C)</p> Signup and view all the answers

<p>It causes the misfolding of the prion protein. (A)</p> Signup and view all the answers

<p>Severe fever. (A)</p> Signup and view all the answers

<p>From infected blood and certain foods. (D)</p> Signup and view all the answers

Group of rare, progressive neurological disorders caused by abnormal prion proteins.

Most common type of prion disease, typically affecting people around age 65, and progressing rapidly.

Normal protein that helps brain cells communicate and protects them from damage.

Misfolded proteins that cause prion diseases by self-replicating and damaging brain cells.

Methods used to diagnose sCJD, including MRI brain scans, blood tests, and EEGs.

CJD is caused by a misfolded protein called the prion protein (PrP^c^). This abnormal protein is resistant to enzymes and builds up in the brain, leading to cell death.

There are three types of CJD: sporadic (sCJD, most common), inherited (IPD) and acquired. Sporadic means it develops randomly, inherited is passed down through genes, and acquired is contracted through external factors like surgery or blood transfusions.

CJD symptoms include unsteady gait, jerky muscle movements (myoclonus), blurred vision, problems thinking and planning, memory loss, and behavioral changes. These symptoms often worsen rapidly.

Diagnosis of sCJD often involves brain imaging using an MRI scan, genetic blood testing for the prion gene, and an EEG to assess brain activity.

The misfolded PrP^c^ protein causes prion disease by accumulating inside brain cells and causing them to die. The process is self-amplifying as the abnormal prion proteins convert normal proteins into misfolded ones.