Prionic Diseases

Main Types of Prionic Diseases

Creutzfeldt-Jakob Disease (CJD): A rapidly progressive disease with multiple neurological symptoms, including dementia, muscle weakness, and visual disturbances.
Variant Creutzfeldt-Jakob Disease (vCJD): This is related to BSE (bovine spongiform encephalopathy) or “mad cow disease.” Similar symptoms to CJD but generally begins with psychiatric symptoms.
Kuru: Once common amongst the Fore people of Papua New Guinea, caused by the practice of consuming human brain tissue at funerals.
Fatal Familial Insomnia (FFI): An extremely rare genetic condition causing progressively worse insomnia, leading to hallucinations, rapid weight loss, and ultimately death.
Gerstmann–Sträussler–Scheinker syndrome (GSS): A very rare inherited prionic disease causing progressively worse coordination and cognitive decline.

Symptoms of prionic diseases vary but generally include rapidly progressive dementia, muscle stiffness or twitching, difficulty speaking, and walking or movement problems.
Other signs can include difficulty swallowing, blurred vision or double vision, and loss of bladder or bowel control.
Some people may also experience psychiatric symptoms such as depression, anxiety, or unusual sensations.

Prions can cause disease by changing the shape of normal proteins within cells, which can then aggregate and cause damage and death to the cells.
Once symptoms begin, prionic diseases typically progress rapidly, leading to severe disability and often death within a year in diseases like CJD.
There are currently no treatments that can cure prionic diseases, though some medications may help to manage symptoms.

The risk of acquiring a prionic disease can be reduced by not consuming contaminated meat, and ensuring that medical instruments are properly sterilised.
Genetic counselling can provide individuals who have a family history of inherited prionic diseases with information about their risk.
There is ongoing research into the development of a vaccine, but as yet there is no way to prevent prionic diseases.