PRIONS

 Prion disease is a rare neurodegenerative disorder that affects humans and animals, causing brain damage and dementia. It affects about 1 in 1 million people worldwide and is terminally ill. Healthcare providers focus on treatments, including medication, to manage symptoms and help people cope with the changes it causes.

Prion disease can be acquired through genetic mutations (familial prion disease) or exposure to prion infection (acquired prion disease). Sporadic prion diseases, such as Creutzfeldt-Jakob disease (CJD), fatal insomnia, and variable protease-sensitive prionopathy, occur when normal proteins turn into prions for no known reason. Familial prion diseases develop when the PRNP gene mutates, creating an abnormal protein. There are over 50 different PRNP mutations that can cause different inherited prion diseases.

Acquired prion disease can happen if people are exposed to contaminated food or medical equipment. Kuru, the first transmissible neurodegenerative disorder identified and studied, occurred among the Fore people of Papua New Guinea and is believed to be transmitted from person to person by ritual cannibalism.

All prion diseases are deadly, meaning people die from the disease. Symptoms vary depending on the specific disease and how it affects the brain. Common prion disease symptoms include ataxia, aphasia, confusion, insomnia, memory loss, thinking and judgment issues, hypokinetic movement disorders, myoclonus, personality changes, and psychological issues like anxiety, depression, and visual hallucinations.

In summary, prion disease is a rare and terminal illness that can be caused by genetic mutations or exposure to contaminated food or medical equipment. Healthcare providers focus on treatments, including medication, to manage symptoms and help people cope with the changes it causes.








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