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PRION DISEASES

Prion is a protein without genetic material.    

Prions are tiny proteins in your brain that do not act the way they should. They fold over the wrong way, spread, and then cause other proteins to form the same incorrect shape. These badly shaped prions build up and form clumps in your brain. Then they kill neurons or brain cells that control your memory, balance, and movement.

  

Your immune system cannot fight them off, therefore, more healthy cells die.    

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If the prion is folded abnormally, it affects the structure of the brain and causes deadly diseases. Creutzfeldt-Jakob disease (CJD) is the most common. It usually affects people around age 60. Variant Creutzfeldt-Jakob disease can affect younger adults. Such

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diseases spread rapidly and are usually fatal. They do not replicate in the host but stimulate abnormal behavior in the body cells.  

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If the prion is folded abnormally, it affects the structure of the brain and causes deadly diseases. Creutzfeldt-Jakob disease (CJD) is the most common. It usually affects people around age 60. Variant Creutzfeldt-Jakob disease can affect younger adults. Such diseases spread rapidly and are usually fatal. They do not replicate in the host but stimulate abnormal behavior in the body cells.  

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TYPES OF PRION DISEASES

 

  • CJD. A person can inherit this condition, in which case it's called familial CJD. Sporadic CJD, on the other hand, develops suddenly without any known risk factors. Most cases of CJD are sporadic and tend to strike people around age 60. Acquired CJD is caused by exposure to infected tissue during a medical procedure, such as a cornea transplant. Symptoms of CJD (see below) quickly lead to severe disability and death. In most cases, death occurs within a year. 

  • Variant CJD. This is an infectious type of disease that is related to “mad cow disease.” Eating diseased meat may cause the disease in humans. The meat may cause normal human prion protein to develop abnormally. This type of disease usually affects younger people. 

  • Variably protease-sensitive prionopathy (VPSPr). This is also extremely rare, it is similar to CJD but the protein is less sensitive to digestion. It is more likely to strike people around age 70 who have a family history of dementia. 

  • Gerstmann-Sträussler-Scheinker disease (GSS). Extremely rare, but occurs at an earlier age, typically around age 40. 

  • Kuru. This disease is seen in New Guinea. It's caused by eating human brain tissue contaminated with infectious prions. Because of increased awareness about the disease and how it is transmitted, kuru is now rare. 

  • Fatal insomnia (FI). A rare hereditary disorder causing difficulty sleeping. There is also a sporadic form of the disease that is not inherited. 

 

SYMPTOMS

 

Signs of prion diseases include sudden changes in your mood, memory, and movement, including:   

  • Anxiety or depression   

  • Balance problems   

  • Behavior or personality changes   

  • Dementia   

  • Memory loss   

  • Muscle control loss, like sudden jerks or twitches   

  • Seizures   

  • Slurred speech   

  • Trouble swallowing   

  • Unsteady walk   

  • Vision problems 

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HOW ARE PRION DISEASES DIAGNOSED?  

 

Prion diseases are confirmed by taking a sample of brain tissue during a biopsy or after death. Healthcare providers, however, can do a few prior tests 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. 

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The tests include: 

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  • MRI (magnetic resonance imaging) scans of the brain 

  • Samples of fluid from the spinal cord (spinal tap, also called a lumbar puncture) 

  • Electroencephalogram, which analyzes 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 

TREATMENT  

 

Prion diseases cannot 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. 

 

CAN PRION DISEASES BE PREVENTED?  

 

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 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. 

 

KEY POINTS ABOUT PRION DISEASES  

 

  • Prion diseases are exceedingly rare. 

  • Symptoms can progress rapidly requiring help with daily needs. 

  • Prion diseases are always fatal. 

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