Prion Diseases Treatment News and Information

What are Prion Diseases and What Causes Them? (Definition)

Prion diseases or transmissible spongiform encephalopathies (TSEs), comprise several conditions which affect the nervous system in both humans and animals (neurodegenerative disorders). In humans, these conditions impair brain function, causing changes in memory, personality, and behavior.

The causative agents of TSEs are believed to be prions. A prion is a type of protein that can trigger normal proteins in the brain to fold abnormally. The abnormal folding of the prion proteins leads to brain damage and the characteristic signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal. Prion diseases are sometimes spread to humans by infected meat products.

According to the U.S. National Library of Medicine, between 10 and 15 percent of all cases of prion diseases are caused by mutations in the PRNP gene. These forms of prion diseases are classified as familial, as they are passed down in families. Familial prion diseases include:

  • Familial Creutzfeldt-Jakob disease (CJD)
  • Gerstmann-Sträussler-Scheinker syndrome (GSS)
  • Fatal familial insomnia (FFI)

The remaining 85 to 90 percent of cases of prion diseases are either sporadic or acquired. People with sporadic prion disease have no family history of the disease and no identified mutation in the PRNP gene. Sporadic forms of prion disease include:

  • Sporadic Creutzfeldt-Jakob disease (sCJD)
  • Sporadic fatal insomnia (sFI)
  • Variably protease-sensitive prionopathy (VPSPr)

Acquired prion disease results from exposure to PrPSc from an outside source such as eating beef products containing PrPSc from cattle with prion disease. In cows, this form of the disease is known as bovine spongiform encephalopathy (BSE) or, more commonly, “mad cow disease.”

Approximately 350 new cases are reported annually in the United States.

Prion Disease Symptoms

Symptoms of prion diseases include rapidly developing dementia, hallucinations, confusion, difficulty walking and changes in gait, muscle stiffness, difficulty speaking, and fatigue.

For more information regarding Prion diseases, please visit the Centers for Disease Control website.

See below for updated news and information regarding Prion Diseases including new medical research, treatment options and advancements. 

Latest Prion Diseases News

Alzheimer’s and Concussion-Related CTE May Spread in the Brain Via Common Mechanism

(SOURCE: UC SAN FRANCISCO) - By Pete Farley on December 02, 2016 - Research is First to Suggest That Chronic Traumatic Encephalopathy is a Prion Disease. A shared biological mechanism may drive the progression of both Alzheimer’s disease (AD) and chronic traumatic encephalopathy (CTE), a neurodegenerative condition associated with repeated concussions and brain trauma, according to a new study led by ...
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Possible Urin Test for Sporadic Creutzfeldt-Jakob Disease

(Medical Express) According to a recent article published in Medical Express, researchers from the the MRC Prion Unit at UCL have found that it may be possible to identify whether or not a person has sporadic Creutzfeldt-Jakob Disease (sCJD) by testing their urine for the presence of abnormal prion proteins ...
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Briotech’s Proprietary HOCl Formulation Destroys Deadly Prions

Briotech Inc. recently announced that a study published in the current issue of PLOS Pathogens proves that Briotech's unique formulation of pure hypochlorous acid (HOCl) inactivates infectious proteins, known as prions that cause chronic brain diseases in humans and animals such as Mad Cow, Chronic Wasting, and Creutzfeldt-Jakob Disease ...
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