What's The Deadliest Prion Disease?

By Victoria Simpson on March 16 2020 in Society

Microscopic "holes" are characteristic in prion-affected tissue sections, causing the tissue to develop a "spongy" architecture. This causes deterioration of that "spongy" tissue in the brain. Image credit: Dr. Al Jenny / Public domain
Microscopic "holes" are characteristic in prion-affected tissue sections, causing the tissue to develop a "spongy" architecture. This causes deterioration of that "spongy" tissue in the brain. Image credit: Dr. Al Jenny / Public domain
  • Fatal Familial Insomnia causes you to not be able to sleep, leading to a coma and death.
  • Only about 1 in a million people ever get a prion disease.
  • Mad Cow Disease is a prion disease.

Prion diseases are horrible and always fatal. The blessing is that they are very, very rare, and as such, only around one person in every million ever contracts or develops one. 

The Centers for Disease Control and Prevention defines prion diseases stating,  

“Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of rare progressive neurodegenerative disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response.”

Essentially, prion diseases are illnesses that attack your brain. These diseases trigger normal proteins found in your brain, causing them to fold in abnormal ways, with dire consequences. 

What are some well-known types? Creutzfeldt-Jakob Disease (CJD), Mad Cow Disease (BSE) and Chronic Wasting Disease (CWD) are all examples of prion illnesses. 

You can get a prion disease in various ways. Some, like Mad Cow Disease, are transmitted to humans when you eat infected meat. A person can also develop a prion disease if it runs in their family, as they can be hereditary. Contaminated medical equipment can also cause an infection that leads to one and at other times, people have been known to develop a prion illness for no known reason. 

All prion diseases are devastating and some progress faster than others. Mad Cow Disease has been shown to lie dormant in animals for as long as 2.5 to 5 years, and in humans it can lie undetected for up to a whopping 50 years. Others, however, progress much faster. 

One of the most drastic prion diseases is the aforementioned Creutzfeldt-Jakob Disease (CJD). Aound 70% of all people who develop it die within one year of being diagnosed, and some only last about 6 months. 

How You Get CJD?

According to the CDC, up to 85% of CJD cases have no known source. There is no recognizable pattern of transmission, and it is enough to make you believe in the power and presence of aliens, if you ask some people. 

The other 5% to 15% of people who develop CJD do so because they have inherited it. The inherited forms of CJD include Gerstmann-Straussler-Scheinker syndrome and the awful-sounding Fatal Familial Insomnia. 

How CJD Is Diagnosed?

Doctors can only diagnose CJD conclusively by doing a brain biopsy or an autopsy. This means that in order to get a definitive diagnosis, someone has to surgically remove tissue from your brain, or you do not ever know you have it, and only your friends and family find out after your brain is dissected once you die.

Scientists are currently said to be working on a lab test that can detect CJD, which would obviously be much easier for all involved.

Diagnostic tests, MRIs, spinal fluid tests and electroencephalograms can also lead professionals towards an accurate diagnosis of CJD.  

How It Progresses?

Most often, CJD progresses very rapidly. It destroys brain cells and makes tiny holes in your brain, causing neurodegeneration. People who develop it can experience trouble controlling their body movements, personality changes, depression, memory loss akin to dementia, blurred vision or blindness, trouble swallowing and speaking, and anxiety among other symptoms.

As stated above, most people who have this disease succumb to it within 12 months. Some live for up to two years, and others die within just six months. 

Gerstmann-Straussler-Scheinker Syndrome

This is an inherited form of CJD. You can not get this illness any other way, and thankfully, it is only found in a few families around the world. It often starts with patients experiencing clumsiness and a loss of coordination, followed by mental impairment. It endures about sixty months, or five years. 

Fatal Familial Insomnia

This illness definitely sounds like the title to a horror movie. It is anything but fiction, however.

Fatal Familial Insomnia begins with a general difficulty sleeping, but it soon progresses into physical and mental deterioration. It often begins in middle age, although it can start earlier or late in life. In most, the inability to sleep comes suddenly, and progresses quickly over the following few months.

With this disease, the brain breaks down due to a lack of sleep, and the patient eventually ends up in a coma, and then dies. When alive and the patient does dream, the dreams are vivid. In waking moments, hallucinations can occur and a person’s thinking is generally impaired more as time progresses. Not fun. 

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