The longest someone has lived with a prion disease is 14 to 16 years in cases of Creutzfeldt-Jakob disease (CJD) and with variant CJD (vCJD), survival with prion disease has reached 10 years with experimental treatment.
The longest documented survivors of prion disease include:
Genetic CJD (V180I mutation): A documented case report in the Wiley Online Library details a patient with V180I genetic Creutzfeldt-Jakob disease who survived for 16 years, representing the longest recorded survival duration for any CJD form.
Sporadic CJD: Documented in the European Journal of Neurology via PubMed, a 61-year-old woman with sporadic CJD survived for 14 years, which is the longest known survival period for the sporadic form of the disease.
Variant CJD (vCJD): Jonathan Simms from Northern Ireland lived for 10 years after his vCJD diagnosis (the human form linked to mad cow disease).
He survived significantly longer than the typical one-year expectancy after receiving an experimental continuous infusion of pentosan polysulfate (PPS).
The reason why prion diseases are so scary is because they are misfolded proteins, which can't be killed because they are not actually alive.
Prion diseases cause severe and irreversible brain damage and they are not alive, they act like a chain reaction, they are nearly indestructible and are incurable and 100 percent fatal and always results in death and often within a year of symptoms appearing.
Prion disease has no DNA or RNA like traditional germs like bacteria and viruses that need genetic material to reproduce.
Traditional germs like bacteria and viruses can be destroyed by heat, radiation or chemicals but prion disease cannot.
A prion is also simply a normal protein that has twisted in the wrong shape and you cannot kill something that is not living.
When a misfolded prion touches a normal protein, it also forces that normal protein to change shape and become a prion as well.
And these abnormal proteins clump together and kill brain cells, leaving microscopic holes that turn your brain spongy.
Prions can also survive standard hospital sterilization, being boiled, freezing, radiation and strong disinfectants and surgical tools used on an infected patient often have to be destroyed completely, instead of being reused because ordinary cleaning won't remove the threat.
An infection of prion disease can also lie dormant and show no symptoms for years or even decades and once symptoms of prion disease do finally appear, prion disease moves fast, leading to severe dementia, loss of muscle control and death within a few months and there is no cure or any effective treatment for prison disease.
Another name for prion disease is transmissible spongiform encephalopathy (TSE).
Transmissible spongiform encephalopathy (TSE) is a medical name for prion disease that describes how prion disease can spread and how it makes tiny holes in the brain.
Common types of prion disease include.
Creutzfeldt-Jakob disease (CJD): The most common type of prion disease in people.
Variant CJD (vCJD): Linked to eating meat from cows with "mad cow disease".
Kuru: A rare type of prion disease found in past years in Papua New Guinea.
Bovine spongiform encephalopathy (BSE): Also called mad cow disease, which affects cattle.
Scrapie: A type of prion disease that affects sheep and goats.
Chronic wasting disease (CWD): A type of prion disease that affects deer and elk.
Prion disease is a rare, incurable, and fatal brain disorder that is caused by proteins that misfold and damage the nervous system.
Most cases of prion disease are sporadic and happen by chance with no known cause in about 85% to 90% of cases.
Genetic cases of prion disease are inherited through mutated gene passed down in families which is around 10% to 15% of cases.
Acquired cases of prion disease occurs due to rare exposure to contaminated tissue, food, or surgical gear in less than 1% of cases of prion disease.
Common human forms of prion disease include Creutzfeldt-Jakob disease (CJD), variant CJD, and fatal familial insomnia.
Symptoms of prion disease include rapidly worsening dementia and memory loss, loss of muscle coordination (ataxia), involuntary muscle jerks (myoclonus), vision changes or confusion and personality and behavioral shifts.
For diagnosing prion disease, doctors use Johns Hopkins Medicine guidelines, which include brain MRIs, spinal taps and electroencephalograms (EEGs) to check for rapid cognitive decline.
No cure for prion disease exists, and prion disease always results in death, usually within 1 year of the symptoms starting.
Care for the prion disease focuses on comfort and easing symptoms.