Prions, prion diseases

Prion diseases are rare, progressive and fatal diseases affecting the brain (and more rarely other organs) that can’t be cured at present.

Medical professionals

1 May 2021 | 5 Min. Reading Time

Overview

Prion diseases are rare, progressive and fatal diseases affecting the brain (and more rarely other organs) that can’t be cured at present. They occur when a protein folds into an abnormal form called a prion. Changes in the brain lead to memory and movement disorders, confusion, restlessness and anxiety. These diseases affect animals more often than humans.

Symptoms

Symptoms are similar to those for dementia
Incubation period: months to years

Memory disorders, memory loss
Confusion
Restlessness, anxiety
Irritability, anger
Depression, withdrawal
Sensitivity to noise
Movement disorders
- Tremors
- Muscle contractions
- Muscle weakness
- Scratching
- Uncoordinated movements
- Unsteady gait, tendency to fall
- Immobility

Causes and treatment

Causes

Sporadic prion disease (most common form)
- The cause is not known
- Example: Creutzfeldt-Jakob disease
Familial prion disease
- Inherited disease (genetic)
- Examples: familial Creutzfeldt-Jakob disease, fatal familial insomnia
Acquired prion disease (rare)
- Eating prion-infected beef (mad cow disease)
- Transplant/transfusion by prion-infected donor
- Infected medical instruments (surgery, diagnostics)
Prion disease among animals
- Animal eats another infected animal
- Animal-to-animal transmission through bodily fluids
- Examples: mad cow disease, scrapie (sheep, goats), chronic wasting disease (deer, elk, moose)

Further treatment by your doctor / in hospital

Possible tests

Neurological examination
MRI (magnetic resonance imaging)
Examination of cerebrospinal fluid (lumbar puncture)
Measurement of electrical activity in the brain

Possible therapies

Treatment is dictated by the symptoms
Always fatal (spongy changes in the brain)

Preventive measures by the authorities:

Statutory requirements
- Keeping and feeding of animals, disposal of animal cadavers
- Organ and tissue transplants
- Blood transfusions / blood substitutes
- Medical products (instruments, devices)
Official monitoring of frequency of occurrence and new cases of prion diseases

What can I do myself?

Animals exhibiting unusual behaviour
- Avoid contact, keep your distance
- Contact veterinarian
Grill/cook meat well
Hunters
- Hygiene measures when eviscerating animals
- Don’t eat obviously sick animals
- Have meat tested, if necessary
Keepers of animals: comply with statutory provisions