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

Potential Risks and Causes:

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

Common 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 – therefore often the symptoms are treated and managed.

Medication

.     Pentosan polysulphate  (PPS)

1.)  This drug was used experimentally

2.)  But it did not stop or cure disease

3.)  There are some results that show that it did appear to slow the progression of this disease.

***Other drugs are or will be studied

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