What Are Prion Diseases?

Prions are incorrectly folded proteins that can cause misfolding of other proteins, leading to severe diseases and consequences, and even death. Hence, you must know the symptoms, causes, diagnosis and, most importantly, prevention techniques of these diseases to protect yourselves from their severity.

This is a fatal, easily transmissible, and untreatable neurodegenerative disorder that are also rare. Prion diseases can occur in both humans and animals. There are a variety of prion diseases that you must be aware of to take the necessary precautions.

What Are the Types of Prion Diseases?

The types of Prion diseases that occur in humans are as follows:

Creutzfeldt-Jakob disease (CJD)
Gerstmann-Straussler-Scheinker syndrome (GSS)
Kuru
Fatal familial insomnia (FFI)
Variant Creutzfeldt-Jakob disease (vCJD)

Once this disease starts developing in your body, you can notice several symptoms for the same.

What Are the Signs and Symptoms of Prion Diseases?

The symptoms indicating prion disease are as follows:

Difficulty in thinking: You might face difficulty in remembering and judging things correctly.
Spasms in involuntary muscles: There can be occasional spasms in the involuntary muscles.
Loss of muscle coordination: You might face a lack of coordination between your brain and muscles.
Troubled sleeping: You might experience insomnia.
Slurry speech: Your speech might become slurry with passing days, indicating towards this disease.
Confusion: You might experience confusion in your daily work owing to improper functioning of your brain.
Personality changes: There can be noticeable changes in your behaviour, such as agitation, apathy and depression.
Impaired vision: There can be a loss of vision due to the development of this disease.
Dementia: You can have dementia due to prion disease.
Seizures: You can encounter sudden and unexplained electrical disturbances in the brain leading to behavioural changes and mood swings.
Difficulty in swallowing: You might face difficulty in swallowing your food due to this disease.
Difficulty in balancing: You might lose your body’s balance frequently when suffering from this disease.

What Are the Causes of Prion Diseases?

The primary cause behind the development of prion disease is the misfolding of prion proteins which are found in nerve and brain cells. Although these misfolded proteins normally get recycled, they can accumulate in the brain at times due to several reasons.

The exact function of this protein is still unknown, yet scientists believe that they play a crucial role in signal transduction. Hence, misfolding can result in the disruption of signal conduction between the brain cells leading to their death.

However, the misfolded prion protein also stimulates other proteins to misfold, which leads to the formation of plaque. This ultimately causes small holes in the brain resulting in physical and mental impairment and, ultimately, death.

Additionally, there are several factors that increase the risks of the development of prion disease.

What Are the Risk Factors Involved in Prion Diseases?

Below are some risk factors that increase the chances of occurrence of a prion disease:

Age: The chances of development of this disease increase considerably with an increase in age.
Genetics: There are high chances of occurrence of this disease if anyone from your family has a history of this condition.
Medical equipment: One of the prominent causes of this disease is the use of unsterilized medical equipment during treatment.
Animal products: If you consume dairy products that contain prions, there can be a high chance of their transmission to your body.

So, you must not delay in diagnosis after starting to notice its symptoms to prevent its severity.

How Prion Diseases Are Diagnosed?

Doctors often suggest the following methods of diagnosis of prion diseases:

MRI: An MRI of your brain helps doctors to clearly visualise and accordingly detect if prions are present.
EEG: This is used to detect the electrical activity that is going on in your brain. If your brain has prion accumulation, shorter waves are produced.
CSF testing: The cerebrospinal fluid is taken out using a needle and tested to check the presence of prions or markers associated with neurodegeneration.
CT scan: During this test, the laboratory technicians take X-rays of your brain from different angles to quickly detect the disease.

Once confirmed, you must proceed with treatment to check its spread and avoid severe complications.

What Is the Treatment Method Followed for Prion Diseases?

There are no such treatments available for the treatment of prion disease. However, doctors can suggest the following methods to mitigate the symptoms:

Assistance with daily activities: With the progress of the disease, the body becomes weak and needs assistance to perform daily activities.
Proper medicines: The doctor prescribes several medicines, such as painkillers, antipsychotic drugs, sedatives, and antidepressants, to deal with body aches, insomnia, and mood swings.
Providing hydration and nutrients: The patients are supplied with feeding tubes and IV fluids with advancement of the disease.

However, you can take specific precautionary measures to curb this disease at its early stages.

What Are the Preventive Measures for Prion Diseases?

You can take the following precautions to stay safe from the development of prion disease in your body:

Not allowing people with prion disease to donate blood or body tissues.
Defining strict regulations on cattle import from regions that are prone to BSE.
Proper disposal of medical equipment, preventing the transmission of such diseases.
Sterilising the medical equipment properly prior to their use.
Strictly prohibiting the use of the brain and spinal cord in food preparation for both humans and animals.

Additionally, timely medical attention can provide you temporary relief from the symptoms of this disease.

When to See a Doctor for Prion Diseases?

You must visit a doctor as soon as you start noticing the symptoms of prion disease. This will help you to get the proper medication for temporary relief from body aches, mood swings, depression and insomnia.

Since no such treatment to curb the misfolding of prions is available yet, it becomes necessary to strictly adhere to the preventive measures. This keeps a check on its occurrence and allows individuals to avoid complications of this disease.

Frequently Asked Questions

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What are some genetic prion diseases?

Some genetic prion diseases are:

Gerstmann-Sträussler-Scheinker Syndrome (GSS)
Familial Creutzfeldt-Jakob Disease (fCJD)
Fatal Familial Insomnia (FFI)

What are some sporadic prion diseases?

This type includes diseases such as:

Sporadic Creutzfeldt-Jakob Disease (sCJD)
Sporadic Fatal Insomnia (sFI)

What is acquired prion disease?

This type of prion disease includes:

Kuru
Variant Creutzfeldt-Jakob Disease (vCJD)
Iatrogenic Creutzfeldt-Jakob Disease (iCJD)

Are prion diseases rare?

Yes, prion diseases are highly rare. The most common prion disease is Sporadic Creutzfeldt-Jakob which affects only about one person among one million people across the world in a single year.