Prion Disease - trigger normal proteins in brain

A prion is a type of protein that can trigger normal proteins in the brain to fold abnormally. Prion disease can affect both humans and animals and are sometimes spread to humans by infected meat products.

Prion Disease - trigger normal proteins in brain

Author: Beenish Rehman

The most common form of prion disease that affects humans is Creutzfeldt-Jakob disease (CJD). Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, fatal brain disorder. It affects about one person in every one million per year worldwide. CJD usually appears in later life and runs a rapid course.

Causes of Prion Diseases

  • Prion diseases occur when normal prion protein, found on the surface of many cells, becomes abnormal and clump in the brain,
  • It is causing brain damage.
  • This abnormal accumulation of protein in the brain can cause memory impairment,
  • It causes personality changes,
  • It also causes difficulties with movement.
  • Experts still don’t know a lot about prion diseases, but unfortunately, these disorders are generally fatal.

Risk factors

  • Family history of prion disease
  • Eating meat infected by “mad cow disease”
  • Infection from receiving contaminated corneas or from contaminated medical equipment

Symptoms for prion diseases:

  • Rapidly developing dementia
  • Difficulty walking and changes in gait
  • Hallucinations
  • Muscle stiffness
  • Confusion
  • Fatigue
  • Difficulty speaking

Diagnosis of Disease

Prion diseases are confirmed by taking a sample of brain tissue during a biopsy or after death. Healthcare providers, however, can do a number of tests before to help diagnose prion diseases such as CJD, or to rule out other diseases with similar symptoms. Prion diseases should be considered in all people with rapidly progressive dementia.

Treatment of prion diseases

Prion diseases can’t be cured, but certain medicines may help slow their progress. Medical management focuses on keeping people with these diseases as safe and comfortable as possible, despite progressive and debilitating symptoms.

Prevention of Prion diseases

  • Properly cleaning and sterilizing medical equipment may prevent the spread of the disease.
  • If you have or may have CJD, do not donate organs or tissue, including corneal tissue.
  • Newer regulations that govern the handling and feeding of cows may help prevent the spread of prion diseases.

Living with prion diseases

  • As prion diseases progress, people with these diseases generally need help taking care of themselves.
  • In some cases they may be able to stay in their homes, but they eventually may need to move to a care facility.