Prion Disease - Types, Symptoms, Diagnosis, and Treatment

What Is Prion Disease?

Prion disease is a collection of disorders that affect both human and animals neurological systems. These disorders decrease brain function in people, resulting in changes in behavior, memory, and personality, as well as a deterioration in intellectual function (dementia) and aberrant movements, specifically trouble coordinating motions (ataxia). Prion disease symptoms usually appear in adulthood and deteriorate over time, eventually leading to death within a few months or several years.

What Is a Prion?

The name prion was first used to describe an unknown infectious agent that causes numerous neurodegenerative disorders in mammals, including Creutzfeldt-Jakob disease (CJD) in humans. The term comes from the phrase "proteinaceous infectious particle," which alludes to the once-controversial idea that the infectious agent causing such diseases is made entirely of protein and has no nucleic acid genome.

A prion is a protein that causes sickness in both animals and humans by usually causing healthy proteins in the brain to fold improperly. Because prion proteins are only proteins with no genetic material, they operate differently from bacteria and viruses. When a misfolded prion infects a healthy person (perhaps through infected food), it changes properly folded proteins into the disease-associated version. Nobody knows how this happens right now.

What Are the Types of Prion Diseases?

Prion disease can affect humans and animals alike. The following are some examples of prion illnesses.

Prion disease in humans:

• Creutzfeldt-Jakob Disease (CJD)- This disease can be inherited, in which case it is known as familial Creutzfeldt-Jakob disease. Sporadic Creutzfeldt-Jakob disease, on the other hand, appears out of nowhere and has no known cause. The majority of Creutzfeldt-Jakob disease cases are sporadic, striking persons around the age of 60. The symptoms quickly progress to severe impairment and death.

• Variant Creutzfeldt-Jakob Disease (VCJD)- This is a contagious condition connected to "mad cow disease." Humans may contract the sickness by eating infected meat. The meat may trigger the abnormal development of normal human prion protein. Younger persons are more likely to be affected by this sort of condition.

• Gerstmann-Straussler-Scheinker Syndrome (GSS)- It affects the cerebellum, which controls coordination, balance, and equilibrium in the brain. Gerstmann-straussler-scheinker syndrome is inherited as well. Extremely uncommon; however, it happens at a younger age, usually around the age of 40.

• Fatal Familial Insomnia (FFI)- The thalamus, the portion of the brain that controls the sleeping and waking cycles, is affected by fatal familial insomnia. Insomnia is one of the most common symptoms of this illness. The mutation is dominantly inherited, which means that an affected person has a 50 % probability of passing it on to their children.

• Variably Protease-Sensitive Prionopathy (VPSPr)- It is comparable to Creutzfeldt-Jakob disease, except the protein is less susceptible to digestion. People over the age of 70 who seem to have a family history of dementia are more likely to develop it.

• Kuru- It was discovered among a group of New Guineans. The sickness was spread by ritual cannibalism, which involved eating the corpses of deceased relatives and consuming infectious prion-infected human brain tissue. Kuru is currently uncommon due to increasing awareness of the disease and how it is spread.

Prion Disease in Animals:

• Feline Spongiform Encephalopathy (FSE)- Domestic cats and captive wild cats are both affected by this condition.

• Bovine Spongiform Encephalopathy (BSE)- This kind of prion illness, also known as "mad cow disease," affects cows. Humans who eat meat from bovine spongiform encephalopathy infected cows may get variant Creutzfeldt-Jakob disease.

• Chronic Wasting Disease (CWD)- It gets its name from the fact that sick animals lose a lot of weight. Deer, elk, and moose are among the animals affected by chronic wasting disease.

• Scrapie- Sheep and goats are among the animals affected. Scrapie is the oldest form of prion disease, with reports dating back to the 1700s. Sheep and goats are among the animals affected.

• Ungulate Spongiform Encephalopathy- This prion illness is likewise extremely rare, affecting only exotic species related to cows.

• Transmissible Mink Encephalopathy (TME)- A mink is a tiny mammal that is commonly reared for its fur. They suffer from this extremely unusual kind of prion disease.

What Are the Symptoms Seen in Prion Disease?

Prion disease symptoms include rapid changes in mood, movement, and memory, such as:

• Depression or anxiety.

• Problems with balance.

• Changes in behavior or personality.

• Dementia.

• Memory problems.

• Loss of muscle control, such as jerks or twitches.

• Seizures.

• Speech becomes slurred.

• Having difficulty swallowing.

• Unsteady moves.

• Vision issues.

How to Diagnose Prion Disease?

Prion illnesses can have symptoms that are similar to those of other neurodegenerative disorders, making diagnosis difficult. A brain biopsy performed after death is the only certain way to confirm a diagnosis of prion disease. However, a healthcare expert can identify prion disease using the symptoms, medical history, and a variety of tests.

• Magnetic Resonance Imaging (MRI)- A magnetic resonance imaging (MRI) scan can produce a precise image of the brain. This can aid healthcare professionals in visualizing prion disease-related alterations in brain structure.

• Electroencephalography (EEG)- An electroencephalogram monitors the electrical activity in your brain. Prion disease can cause abnormal patterns, especially in Creutzfeldt-Jakob disease, where transient bursts of enhanced activity are noticed.

• Cerebrospinal Fluid (CSF) Testing- Cerebrospinal fluid can be collected and analyzed for neurodegeneration-related indicators.

• Urine and Blood Tests- A urine test can help the doctor discover disorders like blood clots, renal disease, diabetes, and other metabolic disorders.

• Examinations of the Nervous System and Vision- To check for nerve injury and vision loss.

What Is the Treatment for Prion Disease?

Treatments for prion disorders now only ease the symptoms, and they include:

• Medication- Anti depressants or sedatives can be used to treat psychological symptoms; opiates can be used to treat pain; medications like Sodium Valproate and Clonazepam can be used to treat muscular spasms.

• Hydration and Nutrition- Intravenous fluids or a feeding tube may be required in the later stages of the disease. One may also require a catheter to assist with urine drainage.

• Assistance- People with prion illnesses frequently deteriorate and require assistance to care for themselves. They may require assistance with daily tasks.

Conclusion:

Cleaning and sanitizing medical equipment properly can help prevent the disease from spreading. Do not donate organs or tissue, especially corneal tissue, if you have or are suspected of having the Creutzfeldt-Jakob disease.

New regulations regarding cow handling and feeding could help avoid the spread of prion infections. As prion illnesses worsen, patients often require assistance in caring for themselves. They may be able to remain in their homes in certain situations, but they may eventually need to relocate to a care facility.