The different types of prion disease are human prion disease and animal prion disease.
Animal prion diseases include:
Bovine Spongiform Encephalopathy (BSE): Commonly called mad cow disease, which affects cattle.
Chronic Wasting Disease (CWD): Affects deer, elk, and moose, causing weight loss, staggering, and listlessness.
Scrapie: An old and common disease affecting sheep and goats, causing them to scrape against fences.
Human prion diseases include:
Creutzfeldt-Jakob Disease (CJD): The most common human form, featuring fast memory loss, dementia, and muscle jerks.
Most cases of these prion diseases happen for unknown reasons (sporadic CJD), while others are genetic or rarely acquired.
Variant Creutzfeldt-Jakob Disease (vCJD): A rare form linked to eating beef from cattle sick with mad cow disease.
Fatal Insomnia (FI): A severe condition causing a total inability to sleep, weight loss, and panic, which can be inherited or sporadic.
Kuru: A historic human form found in Papua New Guinea caused by ritual cannibalism.
Gerstmann-Sträussler-Scheinker Syndrome (GSS): A very rare, inherited condition that affects balance, movement, and thinking.
Prion disease is transmitted through contaminated tissues or food or even through medical procedures and genetic inheritance.
Although prion disease does not spread through any casual or social contact.
Acquired transmission of prion disease includes ingestion, medical procedures and biological products.
Prion disease can be acquired through accidental exposure during surgery, through contaminated neurosurgical instruments or even receiving infected tissue grafts like dura mater implants or cornea transplants.
You can also acquire prion disease through eating meat or tissues that are contaminated with abnormal prions, like beef from cattle with bovine spongiform encephalopathy (mad cow disease) that causes variant Creutzfeldt-Jakob disease, or even human brain tissue via ritual cannibalism that causes kuru, and rare cases of transmission of prion disease can occur through human growth hormone extracted from cadavers or specific blood transfusions that are linked to vCJD.
For non infectious origins of prion disease, most cases of human prion disease have no known cause, and a normal protein spontaneously changes into a misfolded prion.
And mutations in the prion protein gene can also be passed down in families, which makes the protein more likely to misfold over time.
The symptoms of prion disease are rapidly worsening dementia as well as loss of balance and muscle twitching.
Prion disease is a rare, fatal brain disorder that causes severe neurological changes as misfolded proteins damage your brain tissue.
Prion disease leads to cognitive and mental changes, movement and physical control symptoms and even vision and specialized symptoms.
The vision and specialized symptoms of prion disease include, vision loos, blurry sight, strange visual disturbances and even rare total blindness.
And even severe insomnia, with the total inability to sleep, which is notable in fatal familial insomnia.
Movement and physical control symptoms of prion disease include:
Ataxia, where you have poor balance and awkward, uncoordinated walking.
Myoclonus, where you have sudden, brief and involuntary muscle jerks or twitching.
Stiffness, in which you have muscle rigidity and slow body movements.
And speech issues, including trouble talking or trouble swallowing as the prion disease gets worse.
And the cognitive and mental changes of prion disease include:
Hallucinations, where you see or hear things that are not there.
Confusion, including disorientation and trouble processing information.
Personality shifts, including sudden agitation, depression, anxiety or even apathy.
And rapid dementia, which includes quick loss of memory, thinking skills and judgement.
Prion diseases spread when misshapen proteins force normal proteins in the body to fold the wrong way, which happens through three main ways: sporadic generation, genetic inheritance, and exposure to infected tissue or medical equipment.
How Misfolded Proteins Spread Inside the Body.
The chain reaction: A single bad protein touches a healthy protein and changes its shape into a prion.
Cell travel: Immune cells like dendritic cells pick up these bad proteins and carry them to lymph nodes and the nervous system.
Brain damage: Over months or years, the prions reach the brain, kill cells, and leave tiny holes that make the brain look like a sponge.
The Three Main Causes of Infection.
Sporadic cases: Most cases happen for no known reason when a normal protein suddenly changes shape on its own.
Inherited cases: People can inherit a changed gene from a parent that makes these bad protein shapes much more likely.
Acquired cases: People or animals can catch the prion disease by eating infected meat, receiving tissue or organ transplants, or using dirty surgical tools.