What are Prions?
- Prions are pathogenic agents that can cause abnormal folding of other proteins. Prion proteins are found prominently in the brain and require specific folding to function properly. Prions are transmissible agents that can cause these proteins to fold incorrectly, leading to neurodegenerative disorders.
How do Prion diseases develop?
- When normal prion proteins fold abnormally and clump together in the brain, the protein clumps can cause brain damage. Brain damage caused by prions can develop into fatal neurodegenerative disorders.
Prion Diseases:
- Prion diseases can be either acquired, genetic, or sporadic. Sporadic diseases are the most common and their causes are unknown, as people contract them without risk factors or gene mutations. They are zoonotic, meaning they can be transferred between humans and animals.
1. Sporadic Creutzfeldt-Jakob Disease: Symptoms of sCJD include memory loss, loss of coordination, anxiety, depression, and impaired thinking. Once symptoms become tangible, the disease progresses quickly and becomes fatal within a few months. This disease can also be contracted by inherited mutations or through medical treatments. Sporadic CJD is the most common, while acquired CJD is the least common due to changes in medical practice that prevent contamination.
2. Variant CJD: Variant CJD is another form of Creutzfeldt-Jakob Disease that is contracted through eating meat infected with BSE (Bovine Spongiform Encephalopathy), a neurological disease caused by prions. BSE typically affects cattle and can cause mental deterioration and ultimate death.
3. Sporadic Fatal Insomnia: Symptoms of Sporadic Fatal Insomnia include difficulty sleeping, weight loss, and a loss of coordination. Ultimately, this disorder leads to unconsciousness and death. Fatal Insomnia can also be contracted through a rare autosomal dominant gene.
4. Gerstmann-Sträussler-Scheinker Syndrome: This disorder is always inherited and is very rare. Symptoms include lack of coordination, dysarthria, rigid muscle tone, and sometimes blindness and deafness.
Treatment:
- While there is no cure for prion diseases, some treatments are being researched that can delay the onset of disease or reduce symptoms.
- Patients with Prion disease are well cared for to make their lives as comfortable as possible as they combat debilitating symptoms.
Important Things to Know:
- Prion diseases are extremely rare, approximately 1-2 cases per 1 million.
- They are always lethal and have weakening symptoms.
- Scientists are working towards developing a treatment.
Sources:
Assessed and Endorsed by the MedReport Medical Review Board
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