prionCreutzfeldt-Jakob Disease is the disease when found in humans that is more commonly known as 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 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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