PRION DISEASE INFECTION (CAN IT BE PREVENTED?)

 

INTRODUCTION:

           Prion illnesses are a group of uncommon neurodegenerative conditions that afflict humans and animals. Long incubation periods, spongiform alterations linked with neuronal death, and a failure to elicit an inflammatory response characterise them. Prions are thought to be the cause of TSEs (transmissible spongiform encephalopathies).

          The functions of these normal prion proteins are still being researched. The aberrant folding of prion proteins causes brain damage and the disease's typical signs and symptoms. Prion illnesses are usually deadly and advance quickly.

WHAT ARE PRIONS?

       Proteinaceous infectious particles are referred to as Prion. Prions are infectious organisms that cause a variety of neurological disorders in mammals, including Creutzfeldt-Jakob disease. Prions cause improper folding of normal proteins in the brain, specifically the misfolding of prion proteins (PrP). Prion affects both humans and animals, and infected meat products can sometimes spread the disease to humans..

 Structure of Prions

HOW CAN YOU GET PRION DISEASES?

          You can get prion diseases in three ways:

·          ACQUIRED:

           Exposure to abnormal PrP from an outside source may occur through contaminated food or medical equipment.

·         INHERITED:

           Mutations present in the gene that codes for PrP leads to the production of misfolded PrP.

·         NATURALLY:

          Misfolded PrP may develop without any known cause.

Prion infection transmission

TPYES OF PRION DISEASES:

           Prion diseases can occur in both humans and animals. Both have disease characterized in different types. Some of them are listed below:

Human prion diseases are:

1.          Fatal Familial insomnia (FFI)
2.         Gerstmann-Straussler-Scheinker syndrome (GSS)
3.          Kuru
4.          Variant Creutzfeldt-Jakob disease (vCJD)
5.          Creutzfeldt-Jakob disease (CJD)

Animal prion diseases are:

1.          Bovine spongiform encephalopathy (BSE)
2.          Chronic wasting disease (CWD)
3.          Scrapie
4.          Feline spongiform encephalopathy (FSE)
5.         Transmissible mink encephalopathy (TME)
6.          Ungulate spongiform encephalopathy

SYMPTOMS:

        The following are some of the symptoms of prion diseases:

• Dementia that progresses quickly
• Walking difficulties and changes in gait
• Hallucinations
• Muscle tenseness
• Personality morphs (Confusion, Difficulty speaking, Depression, Disorientation)
• Fatigue

DIAGNOSIS:

       Prions have symptoms that are similar to those of other neurological illnesses, making them difficult to identify. The only way to diagnose it is by a post-mortem brain biopsy. A medical specialist, on the other hand, can diagnose it using symptoms, your medical history, and many tests. These tests include the following:

• Magnetic resonance imaging (MRI) is a type of imaging that (MRI) A magnetic resonance imaging (MRI) scan can produce a precise image of your brain. This can aid healthcare workers in visualising prion disease-related alterations in brain structure.
• CSF (cerebrospinal fluid) testing CSF can be collected and analysed for neurodegeneration-related indicators. A test for detecting signs of human prion disease was developed in 2015.
• Electroencephalography (EEG) is a type of brain imaging (EEG) This test records your brain's electrical activity

TREATMENT:

        There is presently no treatment for prion illnesses, however medication can help keep the disease at bay. Scientists are still trying to develop a treatment for prion illnesses. Anti-prion antibodies and "anti-prions (Trusted Source)" that prevent the reproduction of aberrant PrP are among the potential therapeutics being studied.

CAN PRIONS BE PREVENTED?

           Properly cleaning and sterilizing medical equipment may prevent the spread of the disease. If you have or may have CJD, do not donate organs or tissue, including corneal tissue. Newer regulations that govern the handling and feeding of cows may help prevent the spread of prion diseases. There’s currently no way to prevent inherited or sporadic forms of prion disease. If someone in your family has had an inherited prion disease, you may consider consulting with a genetic counselor to discuss your risk of developing the disease.