Prion Diseases

By: Madi McGowan and Allie McKeehan

Information:

Pathogen: Abnormally folding proteins called prions.

First Identified: In the 1730's, Scrapie, a disease that affects sheep and goats was discovered.


Symptoms of Prion Diseases:

-Aggression

- Personality Changes

-Anxiety and Depression

-Slurred Speech

-May become mute

-forgetfulness

-everyday skills could be lost

-disturbance in balance

-Jerky Movements

-Shakiness

-Difficulty Swallowing

-Double Vision

-Hallucinations

-Coritical Blindness

-Seizures


How is it Spread?

Prion diseases can be inherited, arise spontaneously, or can be found in contaminated food. They are not contagious.


Forms of these diseases:

-CJD

-vCJD

-Gersman-Straussler-Scheinker Syndrome

-Fatal Familial Insomnia

-Kuru

-BSE

-CWD

-Scrapie


These disease became more well known when Mad Cow Disease and Chronic Wasting Disease became more of an epidemic in cattle and deer in the UK and America. BSE/Mad Cow probably originated by feeding cattle meat/bone meal that contained BSE infected products from spontaneously occuring cases.


Prevention:

-Single-use kits for spinal taps

-Destruction of surgical tools used on the brain or nervous tissue.

-Restrictions on food supply, especially cattle.

-Vaccines and Insulin are monitored


Treatments:

-No cure for Prion diseases, only treatments for some symptoms, and most drugs are experimental.

-Quinacrine reduces growth of abnormal prion proteins.

-Pentosan Polysuphate (PPS) delays onset of prion diseases.

-Some medications can be used to aid with behavioral or sleep disturbances.


CJD Creutzfeldt-Jakob Disease - Mayo Clinic