Picture125226.png This wikispace is dedicated to providing scientific information about the debilitating prion disease fatal familial insomnia which is also known as FFI.


Contents


Page 1: Background Information about FFI
Page 2: Clinical Features and Pathogenesis of FFI
Page 3: Role of the Thalamus with FFI
Page 4: Symptoms of FFI
Page 5: Techniques for Diagnosing FFI and potential Treatments



FFI as a prion disease


To understand all the scientific aspects of this rare and perplexing disease, you need to understand the nature of the primary infectious agent which causes the disease: a prion (pronounced pree-on).




A short video explaining what exactly is a 'prion' and how it causes disease.

What is a prion?


A prion is an infectious agent comprised of a protein. Prions unlike most other proteins exist in two different forms, the normal prion protein and the abonormal 'prion' molecule which has subsequently misfolded and changed whereby forming this second pathogenic conformational shape.

What makes 'the prion' molecule so destructive is the fact that when it comes into contact with a normal prion protein, it induces this normal protein to refold whereby it becomes an abnormal 'prion' molecule. Eventually this becomes a domino effect sufficient enough to cause massive tissue damage particularly of the brain causing what is known as a 'prion disease'.

Fatal Familial Insomnia (FFI) is one such example of a prion disease which also includes Creutzfeldt-Jakob Disease (CJD), Kuru and Bovine Spongiform Encephalopathy (BSE) also known as 'mad cow's disease'.



normal_rogue.gif
An image of the two types of prion molecules: To the left is the normal prion protein and to the right is the misfolded abnormal conformational shape of the infectious 'prion' agent which causes FFI.


Multimedia Sources:


The picture presented on this page was obtained from the following electronic source:
http://www.stanford.edu/group/virus/prion/prion2.html

The video presented on this page was obtained from the following electronic source:
http://www.youtube.com/watch?v=w5aAPEYIL9A