Published on Oct 12, 2022 and last reviewed on Mar 02, 2023 - 5 min read
Prion diseases are a set of unusual neurodegenerative conditions that affect humans and animals alike. Read the article below to learn more about this.
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.
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.
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.
Prion disease symptoms include rapid changes in mood, movement, and memory, such as:
Depression or anxiety.
Problems with balance.
Changes in behavior or personality.
Loss of muscle control, such as jerks or twitches.
Speech becomes slurred.
Having difficulty swallowing.
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.
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.
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.
A medical professional can recognize prion disease by looking at the symptoms, medical background, and results of several testing.
- Magnetic resonance imaging (MRI).
- Electroencephalography (EEG).
- Testing of Cerebrospinal Fluid (CSF).
- Blood and Urine Tests.
- Tests on the nervous system.
Prion disorders are a set of uncommon, deadly brain conditions that can affect both humans and animals. They are brought on by abnormally formed aggregates of typically benign proteins in the brain. Consuming meat from cattle with bovine spongiform encephalopathy, also referred to as "mad cow disease," is linked to one form of the condition known as variant CJD (vCJD).
There is presently no treatment or cure for prion illnesses. Symptom management and quality of life improvement are typically the main goals of treatment. Even though prion diseases have varying survival times. Creutzfeldt-Jakob disease or human prions disease symptoms swiftly cause serious impairment and death. Death usually happens within a year.
Treatments for prion disorders include:
- Medication: Opioids are used to treat pain; antidepressants or sedatives are used to treat psychological symptoms; Sodium Valproate and Clonazepam are used to treat muscle spasms.
- Hydration and nutrition: Intravenous fluids or a feeding tube may be necessary as the condition progresses. To aid with urine outflow, one could additionally need a catheter.
- Assistance: Individuals suffering from prion diseases typically deteriorate and need help taking care of themselves. They could need help with daily tasks
The most reliable technique of disposal for prion-contaminated material is incineration (Incineration is the process of burning at a high temperature). Prion can be destroyed by combustion at 1,000 Celsius.
Yes, it can be detected that prions are rare and challenging to measure in the bloodstream. Protein misfolding cyclic amplification, a method to increase the prions in blood samples (PMCA). In order to make some healthy proteins cluster abnormally and turn into prions, PMCA uses the properties of prions.
It can be challenging to diagnose prion diseases since their symptoms often resemble those of other neurodegenerative conditions. Only a post-mortem brain biopsy can definitively establish a prion disease diagnosis. However, a medical professional can recognize prion disease utilizing the signs and symptoms, medical background, and a range of tests like blood, urine, magnetic resonance imaging, and an electroencephalogram.
Tetracycline and doxycycline have been demonstrated to lower prion levels in vitro and may increase the lifespan of animals with prion disease. However, neither Doxycycline nor Amphotericin B has been shown to be efficient in treating prion disease in humans.
Creutzfeldt-Jakob disease typically manifests later in life and progresses quickly. The typical onset of symptoms happens at the age of 60, and roughly 70 percent of people pass away within a year. People may have memory loss, behavioral abnormalities, poor coordination, and visual impairments in the early stages of the illness. As the condition worsens, there may be involuntary movements, blindness, extremity weakness, and coma, in addition to mental decline.
Prion protein builds up largely in the cerebral cortex in prion disease. Memory loss, personality problems, and movement issues may result from this aberrant protein buildup in the brain.
During prion infections, the immune system seems to act more like a Trojan horse (hidden something) than a defense. Prions appear to consist mostly of a protein, prions proteins, which is identical in sequence to a host-encoded protein, prions proteins, and the particular immune system exhibits a natural tolerance.
The term prions describe aberrant, pathogenic substances that are contagious and have the capacity to cause improper folding of a subset of normal cellular proteins known as prion proteins, which are mostly present in the brain.
An inherited, spontaneous, or infectious disease can be brought on by prions, which are proteins with special characteristics.
Yes, Brain damage and the disease's recognizable signs and symptoms are caused by the prion's abnormally folded proteins. Most proton disorders advance quickly and are always fatal.
One mutated copy of the PRNP gene per cell is sufficient to induce the condition in familial variants of prion disease, which are inherited in an autosomal dominant fashion. The mutated gene is usually inherited from one affected parent by the affected person. Occasionally, a novel gene mutation in a person leads to familial types of prion disease.
Last reviewed at:
02 Mar 2023 - 5 min read
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