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.

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)
  • 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

When to see a doctor?

Further information

Federal Office of Public Health, FOPH (Bundesamt für Gesundheit, BAG)
https://www.bag.admin.ch/bag/en/home.html

National Reference Centre for Human Prion Diseases NRPE (Nationales Referenzzentrum für Prion-Erkrankungen NRPE)
www.neuropathologie.usz.ch

Synonyms

prions, prion diseases, transmissible spongiform encephalopathy (TSE), prion

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CSS offers no guarantee for the accuracy and completeness of the information. The information published is no substitute for professional advice from a doctor or pharmacist.

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