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.