What is Prion Diseases, Causes and Symptoms of Prion Diseases

Prion Diseases

  • Posted on- May 22, 2018
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What are prion diseases?

Prion diseases comprises of several conditions. A prion is a type of protein that triggers normal proteins in the brain and makes it to fold abnormally.

Prion diseases can affect both humans and animals and are sometimes it is spread to humans by infected meat products. The most common form of prion disease that affects humans is Creutzfeldt-Jakob disease (CJD).

What causes prion disease?

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

Who is at risk for prion disease?

Risk factors for prion disease include:

  • If you had a family history of prion disease
  • Consuming meat that is infected by “mad cow disease”
  • Getting infected from contaminated corneas or from contaminated medical equipment

How is prion disease diagnosed?

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

The tests include:
  • MRI (magnetic resonance imaging) scans of the brain
  • Spinal tap, also called lumbar puncture (Liquid samples from the spinal cord)
  • Electroencephalogram that checks brain waves this painless test requires placing electrodes on the scalp
  • Blood tests
  • Neurologic and visual exams to check for nerve damage and vision loss


What are the symptoms of prion disease?

Symptoms of prion diseases include:

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


How is prion disease treated?

Prion diseases can't be cured, but some specific medicines may help slow their progress. The medical management always tries to keep people suffering from these diseases as safe and comfortable as possible, despite of progressive and debilitating symptoms.


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