Prion Diseases – a degenerative brain disorder
Prions are infectious agents, which can fold in various ways structurally. They are transmissible to other prions and lead to diseases, which are similar to viral infections. They are responsible for diseases like mad cow disease, scrapie, Creutzfeldt – Jakob disease (CJD).
A harmless prion is found in every tissue of the body. But they are mostly found in nerve cells and the brain. The problem occurs when the prions are folding, and the body cannot use the prion. However, in normal cases, the body recycles these misfolded prions. But if they are not recycled, they can accumulate in the brain.
Prions can lead to various diseases like scrapie, Creutzfeldt – Jakob disease (CJD) and mad cow disease. So, each of them has different symptoms.
Symptoms of CJD are as follows:
• Walking problems, due to difficulty in balance and problems of co-ordination.
• Slurred speech
• Problems in vision like hallucination and double vision
Symptoms of Scrapie are as follows:
• The affected sheep tends to scrape off their fleeces against trees, fences or rocks compulsively.
• Severe itching sensations
• Excessive lip smacking
• Altered gaits
• Convulsive collapse
Symptoms of mad cow disease are:
• Progressively deteriorating behavior as well as signs of neurological problems
• Increase in aggression
• Slow Ataxia
• Drop in production of milk
Prion diseases are caused when the misfolded prions are built up in the brain and lead other prions to misfold too. This leads to the death of brain cells and release of many more prion cells for infecting other cells of the brain. Finally, many brain cells are killed, and misfolded prions get deposited in the brain. As the brain is damaged, it causes various physical and mental impairments leading to Prion-related diseases like CJD, Scrapie, and Mad Cow disease.
There are no proven cures for Prion-related diseases at present. So at the moment, treatment involves keeping the subject as comfortable as possible and using medicines for reducing the symptoms. For e.g. in the case of CJD, sedatives and anti-depressants can be employed for treating depression and anxiety.
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