In humans this condition is often called Mad Cow Disease

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 becuase of 2 seperate 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]

Classifications

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

Cause:

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

Symptoms

   -     Dementia
   -     Memory loss
   -     Change in personality
   -     Hallucinations
   -     Rigid muscle movement
   -     Decrease balance
   -     Decrease coordination
   -     Seizures

Prevent Transmission

Transmission is possible from one individual to another

   -     human growth hormone products
   -     corneal grafts
   -     other grafts
   -     implants
   -     tissues including brain
   -     blood transfusions
   -     cannibalism (often brain tissue)
   -     other

EKG is helpful in some cases
Fluid from the spine (Cerebrospinal fluid)
MRI

Treatment

No cure is known

.          Pentosan polysulphate   (PPS)

*** this drug was used expirmentially – did not stop or cure disease – did appear to slow progression

Other drugs are or will be studied