Is considered a neurological disease that largely affects the Brain.
It is a very uncommon finding but very serious with a large chance of death and is often incurable.
It is so named because of 2 separate neurologists around 1920 – Creutzfeldt and Jakob.
Occurs in less than 1 person per 1 million per year.
In some cases can be fatal within a few months to weeks.
Some forms may lay dormant for years such as Kuru – [very atypical].
1.) Transmissible spongiform encephalopathy – found in humans
2.) Bovine spongiform encephalopathy – found in humans [Mad Cow Disease]
3.) Chronic Wasting Disease – found in deer and elk
4.) Scrapie – found in sheep
5.) Kuru – human – found in Papua New Guinea possibly transmitted by cannibalism
1.) Prions – infectious agent that are proteins
- Type that affects humans
- Causes refolding of normal or natural proteins into a disease form
- Will continue exponentially
- Prions discovered in 1997
Affect tissues often is the brain – seen in squirrels, cows, and humans
- Memory loss
- Change in personality
- Rigid muscle movement
- Decrease balance
- Decrease coordination
Possible Routes of Transmission
Transmission is possible from one individual to another
- Human growth hormone products
- Corneal grafts
- Other grafts
- Tissues including brain
- Blood transfusions
- Cannibalism (often brain tissue)
EKG is helpful in some cases
Fluid from the spine (Cerebrospinal fluid)
No cure is known
. Pentosan polysulphate (PPS)
*** this drug was used experimentally – did not stop or cure disease – did appear to slow progression
Other drugs are or will be studied