prionCreutzfeldt-Jakob Disease is the disease when found in humans that is more commonly known as Mad Cow Disease. It 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].

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

Possible Routes of 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

Diagnosis

–  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 experimentally – did not stop or cure disease – did appear to slow progression

Other drugs are or will be studied

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